Aplasia cutis congenita is a rare scalp defect with occasional absence of underlying bone and dura. Patients with small defects respond well to conservative management. However, larger defects often can pose management and technical challenge. Successful management of a large, composite aplasia cutis congenita defect using Integra dermal regeneration matrix is presented. At 16 months, the patient showed a stable and supple scalp with evidence of calvarial regeneration.