One case of Congenital Cutis Aplasia is presented. The defect involved includes full-thickness skin defect of scalp and cranium. The patient was treated with debridement of dirty necrosed crust which covered exposed dura mater and with double opposing rotation flap including pericranium for bone regneration. The donor site was covered with skin graft from right thigh. During operation, the superficial temporal arterty was found to be short and weak. And after operation, the margin of flap were congested and finally necrotized. The necrotic wound was treated with conservative management. The vascular impairment is thought to be main course of Congenital Cutis Aplasia. So we conclude that the treatment of choice is conservative management or careful flap surgery for coverage of defect area.