© 2017, Springer-Verlag Berlin Heidelberg. Aplasia cutis congenita (ACC) is a rare congenital malformation of primarily the skin; it is most commonly seen on the scalp but can occur anywhere on the body. The exact etiology is still unclear but there are many suggested causes. Classification systems have been proposed to help categorize patients and assist with treatment. Treatment options are controversial and range from conservative to surgical interventions. We report an extreme case of ACC that included a significant part of the skull. We discuss this case and review salient literature. Although such cases of ACC with bony involvement are rare, this aspect of the pathology should be kept in mind when treating or imaging such patients.