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Von Hipple-Lindua (VHL) syndrome is an autosomal dominant neoplastic disorder in which multiple benign or malignant tumours and cysts develop in central nervous system and visceral organs. Retinal capillary hemangioma is the most frequent and often the earliest manifestation of VHL syndrome. We report a case with multisystemic involvement diagnosed as a case of multiple endocrine neoplasia (MEN) syndrome but the presence of multiple, unilateral peripheral and juxtapapillary retinal capillary hemangioma was able to clinche the definative diagnosis of VHL and thus helped in appropriate management of the patient.