Approximately 25% of hemangioblastomas are a result of Von Hippel-Lindau (VHL) disease, which is inherited in an autosomal dominant manner through a germline inactivating mutation of the tumor suppressor gene VHL on chromosome 3, with subsequent inactivation of the second allele by somatic mutation or gene loss in the neoplastic “stromal” cell. The gene discussed is VHL; the disease is hemangioblastoma.