Abstract
BACKGROUND: To describe a case of von Hippel-Lindau disease with peripheral retinal nonperfusion. CASE PRESENTATION: A 66-year-old female with known cerebellar and midbrain hemangioblastomas was evaluated for a retinal hemangioblastoma in the right eye. She underwent widefield fluorescein angiography, which showed hyperfluorescence localized to the hemangioblastoma surrounded by peripheral retinal nonperfusion in the same quadrant. CONCLUSIONS: Further widefield imaging studies are required to determine if peripheral retinal nonperfusion is a common finding in von Hippel-Lindau disease.