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.
Keywords: Eye; Fluorescein angiography; Hemangioblastoma; Nonperfusion; Tumor; von Hippel-Lindau.