Aplasia cutis congenita is a rare disease of unknown etiology, usually affects the scalp, but any part of the body may be affected, full thickness loss is extremely rare. We report a case of a boy born with a large scalp and skull defect measuring 8*9 cm without associated anomalies.
After 2 years follow up with conservative treatment, the patient now is without neurodeficit, with approximately healing of affected skine and small reduction of the bone defected size.