Neonatal compartment syndrome is extremely rare, but several cases associated with compound presentations have been reported [
6,
8,
9]. In all the previously reported cases, the infants had clear clinical manifestations of compartment syndrome immediately after birth [
7,
9‐
12]. Our patient displayed no obvious signs or symptoms of compartment syndrome at birth. Her skin and neurovascular changes developed in the hours after and continued to acutely evolve, prompting urgent escalation of care. The patient’s mother received complete and appropriate prenatal surveillance, and the fetal compound presentation was first documented during labor, seven hours prior to delivery. There was no prolonged labor or arrest of descent. The presumed timing and duration of the injury may explain the unique presentation of our case, which likely represents the initial and early stages of neonatal limb compartment syndrome due to an extrinsic cause. No literature suggests any link between the mother’s medical conditions or medications with fetal malpresentation or compartment syndrome. With timely diagnosis and surgery, our patient had a favorable outcome with normal function and range of motion of the affected extremity at 12 months of age. Clinicians should consider a fetal compound presentation, at any point prior to delivery, a risk factor for neonatal compartment syndrome and should closely monitor for signs of it in the hours after birth, even if the initial exam at birth is normal. The presentation of neonatal compartment syndrome can vary in timing but is remarkably consistent with the appearance of a sentinel lesion and other skin changes such as discoloration, desquamation, blistering, or skin necrosis [
5,
6]. The affected limb also becomes tender, tense, swollen and eventually progresses to motionlessness and pulselessness [
6,
10]. The normal timeline for neonatal compartment syndrome has not been well documented due in part to its extreme rarity, but our case offers new insight. Prompt recognition and treatment with fasciotomy are critical in these patients as irreversible muscle and nerve damage occurs within 8 h of ischemia [
10]. Swift intervention also prevents complications such as Volkmann contractures, bone development abnormalities, neuropathies, scar contractures, and the need for amputation [
6,
7,
9]. Ragland et al. compiled a case series of 24 patients who suffered from neonatal compartment syndrome. Of the 24 patients, only one underwent fasciotomy within the first 24 h of life and was the only patient without subsequent complications [
4]. Diagnosis remains a challenge due to its rarity and because neonatal compartment syndrome can mimic other diseases, such as cellulitis [
10]. As such, delay in diagnosis is the most common cause for delayed surgical intervention [
4]. After surgical intervention, our patient was closely followed by the surgical team, occupational therapy, and her primary care provider. Post-operative interventions, including stretching, bracing, scar massaging, and Early Intervention services also likely played a significant role in our patient’s successful recovery. Our case adds to the growing literature associating neonatal compartment syndrome with compound fetal presentation. It also demonstrates the need for swift, surgical intervention and multidisciplinary follow up to ensure normal recovery and development of the affected limb.