A 30-year-old Caucasian man presented with new onset floaters and photopsia. Presence of capillary hemangiomas and angiomatosis retinae in the right eye. Diagnosed with VHL; referred for genetic testing which confirmed diagnosis. History of elevated blood pressure at age 19. History of Pheochromocytoma at age 19; s/p adrenalectomy Family history of brain tumor (uncle and daughter) Systemic Surveillance revealed: Renal cysts (2mm) GIST (gastrointestinal stromal tumor; paraganglioma) No CNS hemangioblastomas Von Hippel Lindau (VHL) results from a mutation in the von Hippel–Lindau tumor suppressor gene on chromosome 3p25.3. (A) Fundus photograph of the right eye with retinal capillary hemangioma with a feeder vessel, draining vessel and exudation in the superior temporal quadrant. (B) Fundus photograph of the right with angiomatosis retinae in the inferior retina. (C) Composite image of the right eye highlighting a capillary retinal hemangioma with a feeder vessel , draining vessel and exudation. (D) Fluorescein angiogram image highlighting capillary retinal hemanigoma.