We report a case of 40- year- old male, presented with complaints of pain abdomen associated with blurring of vision in right eye. Ocular examination showed features of juxta papillary retinal capillary haemangioma in right eye. Comprehensive systemic work up in conjunction with magnetic resonance imaging of abdomen illustrated well encapsulated lesion in supra renal, superior mesenteric and left paraaortic region. Further imaging by I-131 MIBG whole body scan revealed bilateral pheochromocytoma. Here is a report of a rare case of Von Hippel-Lindau disease having juxtapapillary haemangioblastoma in association with pheochromocytoma.