Introduction: Adams-Oliver syndrome (AOS) is a rare congenital anomaly syndromecharacterised by aplasia cutis congenita (ACC) of the scalp and terminal transverse limb defects. It demonstrates variable genetic heterogeneity , and the degree of organ involvement determines prognosis. Early recognition enables appropriate multidisciplinary management and facilitates parental counselling. Case presentation: A male neonate was delivered at 37 weeks of gestation via normal vaginal delivery to fourth-degree consanguineous parents, with a birth weight of 2255g. No resuscitation was required at birth. The pregnancy was complicated by maternal chronic hypertension superimposed with preeclampsia. There was no family history of a similar condition. Physical examination revealed a large scalp and skull defect over the vertex consistent with ACC, hypoplastic terminal limbs, and cutis marmorata telangiectatica congenita, fulfilling the clinical criteria for AOS. Initial investigations demonstrated coagulopathy and hepatic involvement, which resolved with supportive management. Threedimensional computed tomography confirmed a large bony defect at the vertex of the skull vault. Echocardiography revealed biventricular hypertrophy , while abdominal ultrasonography was normal. The neonate was managed with intravenous antibiotics for presumed sepsis and tolerated enteral feeds well. Scalp lesions were managed with debridement of necrotic skull bone and application of a Biodegradable Temporising Matrix (BTM) for staged reconstruction by the plastic and neurosurgical teams. Limb anomalies did not require urgent surgical intervention. Conclusion: AOS requires a high index of clinical suspicion and a multidisciplinarymanagement approach. Prenatal diagnosis may improve outcomes and facilitate early parental counseling.