Aplasia cutis congenita (ACC) of the vertex is a rare and potentially severe malformation. We report the case of a newborn boy presenting ACC of the vertex with a skull defect. Surgical closure of the skin was performed 5 days following birth, which allowed skin healing. The skull spontaneously remodelled perfectly 6 months later. When associated with skull defect, this rare malformation characterized by the absence of skin carries the risk of severe complications such as rupture of the superior sagittal sinus or infections. Based on a review of the literature, we purpose an overview of the medical and surgical management of vertex ACC, which depends on the size of the skin defect and the presence of a skull defect.