Retinal capillary hemangioblastoma (RCH) is a benign vascular tumor originating from the neurosensory retina or optic disc. Although it may rarely occur as an isolated tumor, RCH is typically a hallmark of von Hippel-Lindau (VHL) disease, which results from a VHL tumor suppressor gene mutation located on chromosome 3. VHL disease shows autosomal dominant inheritance and has an approximate incidence of 1/36.000. This disease may also be associated with various benign and malignant tumors other than RCHs. For this reason, besides conducting genetic analysis for the VHL gene mutation, patients with RCHs should be examined for central nervous system and internal organ tumors.