Three patients with developmental abnormalities of the optic disc (two morning glory anomalies and one retinochoroidal-optic disc coloboma) had angiographically documented aberrancies of the carotid circulation including large trunk occlusions, moya-moya-like collaterals, dolichoectasia, and absent ophthalmic artery. Although developmental optic disc abnormalities have been linked with other problems such as basal encephalocele, congenital heart defects, and eyelid hemangiomas, these three patients are the first to our knowledge to have related malformations of the intracranial circulation. We suggest, therefore, that the presence of a congenital optic disc anomaly may herald a similar defect in the cerebral circulation.