Aplasia cutis congenita is a very rare and heterogeneous disease. It is characterized by a localized or extensive skin defect, isolated or associated with damage to the underlying structures, including the bone. The diagnosis is based on the clinical examination. Other abnormalities of various etiologies and severities may be associated with this pathology. Adams-Oliver syndrome is a genetic polymalformative syndrome. The typical form is defined by the presence of Aplasia cutis congenita and limb anomalies. Managing Aplasia cutis congenita, especially in severe cases, is a real challenge. We report a case of a newborn, treated surgically, for extensive occipito-parietal Aplasia cutis congenita. The clinical presentation was suggestive of AdamsOliver syndrome. The evolution was favorable. Describing our experience of managing a case of Aplasia cutis congenita is interesting because: the proposed treatment is very controversial, the rarity and the high morbidity and mortality of Aplasia cutis congenita and Adams-Oliver syndrome. As well as clinical and genetic diversity.

Keywords : Aplasia cutis congenita, Adams-Oliver syndrome, surgery, scalp, treatment