Scrotoschisis is a rare congenital scrotal wall pathology. Its prevalence is unknown. Only 16 cases were reported in the literature [
2,
3]. It is a semi-urgent condition which should be managed immediately if it presents with a testicular torsion. There are many hypotheses for its etiology: Failure of differentiation of scrotal mesenchyme layer results in rupture or avascular necrosis of overlying epithelium leading to scrotal wall defect [
4]. Other reports mentioned external mechanical compression effect due to arthrogryposis [
5], as well as meconium periorchitis [
6]. In our case, we have no obvious cause; there is no evidence of meconium residua and neither is there evidence of traumatism. Iatrogenic injury is a potential cause and could be concurrently associated if obstetrical difficulties were encountered during labor, mostly if completed by cesarean section [
7]. Broad-spectrum antibiotics should be commenced immediately on diagnosis. During a physical examination, it is fundamental to rule out testicular torsion (due to lack of testicular anatomical attachments). A local aseptic dressing procedure is imperative to avoid possible orchitis or peritonitis in case of communicating patent processus vaginalis. A surgical approach depends on the severity and could be a simple dressing with healing by secondary intention [
8] or conventional surgical method in two planes horizontally or vertically after orchidopexy. In our case, we preferred to close in two layers horizontally; the skin defect was mainly horizontal. Postoperative follow-up should not be discontinued until verification of two symmetric, vital, and normally growing testes. Further research is recommended to study the effect of scrotoschisis on fertility.