Aplasia cutis congenita (ACQ is an uncommon condition which is characterized by congenital, localized or widespread absence of skin or scar formation. Lesions can be multiple and may occur on any body surface, although they are mostly seen on the scalp as a solitary lesion. ACC is most often a benign isolated defect; but it can be associated with other physical anomalies or malformation syndromes. Adams-Oliver syndrome is a distinct subtype in which distal limb reduction is found in association with a solitary scalp defect. There is no single underlying cause of ACC; it may reflect a disruption of intrauterine skin development. A 25 day-old boy was referred to our clinic with a thick hemorrhagic crust, and a surrounding hairless atrophic scar on the midline over the skull vertex. He also had hypoplasia of the left 2nd to 5th toes since birth. The rest of the physical examination as well as an ultrasound examination of the brain were normal. Herein we report a rare case of ACC of the scalp accompanied by distal limb hypoplasia, Adamson-Oliver syndrome.