Congenital skin and soft tissue necrosis is a rare condition associated with significant morbidity and mortality in neonates. The authors treated a neonate born with significant skin necrosis of the right forearm. The case report is followed by a literature review and discussion of previously published reports of neonatal skin necrosis. A term female neonate was admitted to our hospital at 24 h of age for skin necrosis of the right forearm with sloughing and edema below the right elbow and contractures of her fingers. Topical treatment with cleansing and antibiotic application was initiated. The LUNA florescent microangiography showed superficial perfusion defects in the arm and dorsum of the hand along with overt ischemia over the dorsal aspect of the forearm. She was treated with intravenous antibiotics following a sepsis evaluation. Subsequently, she developed hypotension treated with fluid boluses, dopamine, and stress dose steroids. Concerns of wound infection and sepsis led to debridement of the necrotic area within the first 24 h post-admission. Wet-to-dry dressing changes using Vashe wound solution were begun postoperatively.; followed by placement of Integra on postoperative day-of-life (DOL) 7; dressing takedown on DOL 12; and autografting of the right hand and forearm with disarticulation of the 4th distal interphalangeal joints and right 5th distal interphalangeal transection on DOL 24. Postoperative dressing care was continued during the remainder of the hospital stay, she remained stable without any further complications and was discharged home on DOL 34 with outpatient clinic follow-up.