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¹Bakırköy Eğitim ve Araştırma Hastanesi Göz Kliniği, İstanbul, Dr.
²Çanakkale 18 Mart Üniversitesi Göz Hastalıkları A.D., Çanakkale, Op.Dr.
³Bakırköy Eğitim ve Araştırma Hastanesi Göz Kliniği, İstanbul, Op. Dr. Wyburn-Mason syndrome which is also called retinoencephalofacial angiomatosis is a rare condition characterized by arteriovenous malformations (AVM)s; the facial structures, orbits, skin and brain. The most common presenting symptoms are impaired vision in orbital AVMs, epilepsy and hemorrhage in patients with cerebral AVMs. The early diagnosis and regular ophthalmological and neurological follow-up of patients is mandatory to prevent fatal intracranial hemorrhages and to perform appropriate approach. The presented case is an 8 year-old boy which had retinal and right hemispheric AVMs and died of a subaracnoid hemorrhage. Keywords : Arteriovenous malformation, vascular dysgenesis, Wyburn-Mason syndrome.