Abstract
Aplasia cutis congenita (ACC) is a rare congenital disease defined by the absence of skin, most commonly on the scalp. While the exact incidence remains uncertain, ACC presents a significant challenge in clinical management due to its variable presentation and associated complications. We present the case of a newborn male with a large scalp defect attributed to ACC, complicated by a life-threatening scalp hemorrhage. Despite challenges in management, including recurrent infections and failed skin grafts, the patient ultimately achieved satisfactory healing following a series of surgical interventions, including local transposition flap procedures. This case underscores the importance of a multidisciplinary approach to managing ACC, tailored to individual patient characteristics and associated risks. While discrete lesions of ACC typically have a favorable prognosis, extensive defects pose significant risks of morbidity and mortality, highlighting the need for careful consideration of treatment options and close clinical monitoring of affected individuals.