A 7-year old male child with an abnormal ophthalmic artery arising from the A1 segment of the anterior cerebral artery is described. The patient suffered growth inhibition which was thought to be caused by a craniobasal cystic lesion affecting the hypothalamus. Preoperative angiograms revealed no vascular abnormalities. The right ophthalmic artery, however, could not be identified. During resection of a large arachnoid cyst the ophthalmic artery was found to arise from the A1 segment of the anterior cerebral artery. To the best of our knowledge, this exact anomaly has not previously been reported. The clinical, neuroradiological and intraoperative findings are presented.