Müllerian duct anomalies affect 2-3% of women [
10]. The combination of uterus didelphys -class III of American Fertility Society –AFS- classification [
11] and obstructed hemivagina was described the first time in 1922 [
12], then in 70’s Herlyn, Werner and Wunderlich reported other similar cases associated with renal anomalies [
1,
2]. In 1983 this condition was identified as a syndrome characterised by the triad of uterus didelphys, blind hemivagina and ipsilateral renal agenesis [
13]. Since 2007 this syndrome was named with the acronym of OHVIRA syndrome (obstructed hemivagina, ipsilateral renal anomaly), including two of the three of defects of the HWWS, which consists of different uterus anomalies (uterus didelphys or uterus septum) and renal anomalies as well as renal agenesis or polycystic kidney [
14‐
16]. The occurrence of the HWWS seems to be 0.1-3.8%, and it is probably underestimated [
3]. HWWS was included in the class U3B uterine anomaly, class C2 cervix anomaly, and class V3 vaginal anomaly according to the classification of ESHRE/ESGE [
17]. The exact etiology of HWWS is still unclear, but it may be related to an abnormal development of the para- and mesonephric ducts. Didelphys uterus results from the failure of the fusion and differentiation of the Müllerian ducts during the eight week, which should give rise to cervix and uterus [
18]. The Wolffian ducts give rise to ureters and kidneys, so when one of these is absent, the ureter and the kidney cannot fuse and the ipsilateral Müllerian duct is lateralized moving away from the urogenital sinus, causing the formation of a blind sac that will correspond to the blind or obstructed hemivagina. The distal portion of the vagina originating from the urogenital sinus is not affected and develops normally. Patients affected by HWWS have no specific symptoms until puberty [
5], then they typically present acute pelvic pain, dysmenorrhea, presence of pelvic mass, recurrent urinary tract infections and urinary retention. The diagnosis usually comes in the adolescence, a few years after the menarche, rarely at the birth or during pregnancy [
19,
20]. In fact there is only a case described in literature by Wu et al. of a neonate diagnosed for HWWS presenting as a mass prolapsing per vaginum [
21]. Clinical suspicion and early diagnosis are imperative to making a timely treatment to prevent complications such as infertility, endometriosis, pelvic adhesions, pyosalpinx, hemato or pyocolpos, and other obstetrics problems [
22]. MRI was considered the gold standard for the diagnosis, but the 3d ultrasound plays always a major role for the identification of uterine anomalies [
23]. Diagnostic laparoscopy in HWWS should performed only when the diagnosis by imaging is not clear or when MRI is not available [
5,
24]. Twenty-five percent of women affected by Müllerian ducts anomalies presents obstetric complications such as recurrent miscarriage, abnormal fetal presentation, postpartum hemorrhage, retained placenta, fetal mortality, fetal growth restriction, premature rupture of membranes [
7]. Obstetric outcome of HWWS women was studied after different surgical treatments. These may consist of conservative treatments like the desobstruction of hemivagina, the therapeutic drainage of hematocolpos, the vaginal septotomy and marsupialization; or less conservative interventions like laparoscopic hemi-hysterectomy associated or not with ipsilateral salpingectomy [
25‐
28]. The best treatment of HWWS is controversial but most of the authors conclude that an explorative laparoscopy with vaginal septotomy and drainage of hematocolpos is enough to restore the functionality of both part of the uterus, avoiding hemi-hysterectomy [
29,
30]. However, HWWS has good obstetric prognosis: 87% of pregnancy rate [
27], approximately 62% positive obstetric outcomes without complications during delivery [
5,
7]. Haddad reported the reproductive performance of 42 patients with obstructed hemivagina, 9 of whom had 20 pregnancies with 69% of live births [
31]. Heinonen reported the reproductive performance and obstetric complications of 49 patients affected by didelphys uterus finding that the incidence of primary infertility was not significantly increased in these women. The rate of spontaneous miscarriage was 21%, not significantly different from the general population (15-20%). Preterm birth took place in 24% of all parts. This rate is higher than that of the general population (9-10%). Instead the caesarean sections rate is 84% and it reflects the high incidence of the breech presentation (51%) [
29,
32]. In the presentation of this case we drew attention to its rarity because it involves the left part of the body, while usually is the right side affected [
28,
33,
34] and the association with a malformation of the skeleton of the lower limbs, ectrodactyly of the right foot, was not ever been reported in literature; we found only a case report which described the combination of HWWS and lumbar scoliosis [
35] and we are not sure if the skeletal involvement could be only an incidental finding. However, not all authors agree that it is a rarity the involvement of the left side: Yavuz et al. presented a case series of 13 women affected by HWWS and 6 of them had anomalies on the left side of the uro-genital tract [
36]. According to the classification proposed by Zhun et al, this case belonged to class 1.1 of HWWS with a complete vaginal obstruction for a blind hemivagina [
37] and she had no fertility problems following surgery.