Optic Nerve Head Hemangioma - An Unusual Presentation of Von Hippel- Lindau Disease

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Von Hippel–Lindau (VHL) disease is an autosomal dominant neoplastic disorder in which multiple benign or malignant tumours and cysts with specific histopathologic features develop in the tissues of the central nervous system and in visceral organs. Retinal capillary hemangioma is the most frequent and often the earliest manifestation of VHL disease. However, optic nerve head hemangioma is a rare manifestation. We report a case of optic nerve head haemangioma in an Asian male which on extensive evaluation proved to be a case of VHL disease associated with pheochromocytoma.