Incidence: 1 in 31,000 to 36,000 live births. ≈ 30% of patients with cerebellar hemangioblastoma have VHL 1).
Von Hippel-Lindau disease has an incidence of one in 36,000 births. There is over 90% penetrance by the age of 65.
Age at diagnosis varies from infancy to age 60–70 years, with average patient age at a clinical diagnosis of 26 years.