Aplasia cutis congenita is an extremely rare disease characterized by congenital localized or widespread absence of skin. We present a catastrophic case of aplasia cutis congenita from a pregnancy complicated by elevated maternal serum and amniotic fluid -fetoprotein levels with a brief review of literatures. Delivery occurred at 38 weeks by cesarean section due to larger fetal head. The neonate lacked more than 90% of his skin and died in soon.