The association of aplasia cutis congenita of the scalp with distal malformations of the limbs is known as Adams-Oliver syndrome. Other reported associations include palatine or auricular malformations, cardiovascular alterations, and spina bifida. Multiple hereditary patterns have been described for this condition, and sporadic cases have also been reported. We report an infant with Adams-Oliver syndrome associated with intracranial calcifications in whom no evidence of intrauterine infection could be demonstrated.