A 1-day-old boy with the characteristics of Adams-Oliver syndrome was presented. Adams-Oliver syndrome has a wide spectrum of anomalies ranging from aplasia cutis congenita, cutis marmorata telangiectatica congenita and transverse limb defects to lethal anomalies. Our patient had aplasia cutis congenita with scalp, skull and dura defect. He had also a large dura defect with herniation of brain tissue. Besides these he had bilateral clubfoot, cortical fissure and nail hypoplasia in the hands, scrotal hyperpigmentation and generalized cutis marmorata telangiectatica congenita. He was operated on the 3rd day of life. The herniated brain tissue was resected and the dura was repaired with a synthetic dural graft.