A newborn baby boy presented with a full thickness defect of the scalp, skull, and dura measuring 6 x 7 cm caused by aplasia cutis congenita. Full thickness loss is extremely rare and to our knowledge this case is the twenty-first reported. It has an established mortality of up to 55%. An encephalocele forced us to do an emergency operation with a single large scalp flap based on the supratrochlear and superficial temporal arteries. After revision the child is now 9 months old and progressing well. There are several ways to treat these rare and delicate cases.