Aplasia cutis congenita with or without congenital anomalies is a rare congenital disorder most commonly involving the skin of the scalp, as well as the skull and dura.The etiology is uncertain, and several theories, including vascular accident intrauterine period, vascular anomaly, intrauterine infection, teratogen, and aminiotic adhesion, have been proposed. One theory is that lesions of the scalp are usually caused by vascular anomalies.The authors report on a patient with aplasia cutis congenita presenting with a huge skin and skull defect combined with aplasia of the superficial temporal artery, which was thought to be the etiology.