Aplasia cutis congenita is a rare congenital abnormality first described in 1767 by cordon. It mostly appears as a solitary lesion involving various layers of the skin and sometimes the bone on the scalp, limbs or abdomen. Genetics, environmental and exogenous causes have been implicated as potential causes. Only about 500 cases have been reported globally as of 2013. Two cases of Aplasia Cutis Congenita (ACC) who presented with scalp and bone defects at birth are reported, one in a syndromic child delivered to a consanguineous family, with associated cardiac, skin and nail anomalies (likely Adams Oliver syndrome) and the other as an isolated scalp lesion. Both were large defects managed conservatively by a multidisciplinary team. The challenges of investigating and managing such complex scalp anomalies in sub-Saharan Africa are highlighted.