Aplasia cutis congenita (ACC) is a rare congenital defect of skin and subcutaneous tissue, more rarely of periosteum, skull and dura. The lesions can involve any location, but most common are scalp defects. We report on the successful treatment of three large defects of the scalp with skull involvement in a newborn girl by early debridement and defect closure with two opposed scalp rotation flaps and an occipital split-thickness skin graft.