A 15-year-old girl presented with a 2-month history of decreased vision and a dark spot in the central vision in her right eye. She had a history of optometric examinations with no known ocular disease. Visual acuity with correction was 20/40 in the right eye and 20/20 in the left eye. Anterior segment examination of both eyes was unremarkable, and the retina in the left eye appeared normal. In the right eye, there was an area of superior macular whitening with associated intraretinal hemorrhages and exudates. Fluorescein angiography showed abnormally dilated and tortuous retinal vessels, irregular capillary branching patterns, and bulblike telangiectasis with late leakage in the superior macula. Optical coherence tomography showed disorganized and hyperreflective inner retinal thickening (Figure, A) and intraretinal and subretinal fluid in the central macula. On review of systems, the patient noted a long-standing growth on her cheek, which on examination was a 4 × 3-mm exophytic, brownish-red vascular papule (Figure, B), as well as a history of lightly pigmented patches of skin and multiple subungual fibromas on her toes. Systemic blood pressure was normal.