von Hippel–Lindau disease is diagnosed (6) in a patient who fulfills any one of the following four conditions: (1) two or more CNS hemangioblastomas; (2) one CNS hemangioblastoma and a disease-associated visceral tumor (i.e., RCC, PCC, pancreatic tumor or cysts, or broad ligament cystadenomas); (3) a family history of VHL disease and one of the following: (a) retinal angioma, (b) spinal or cerebellar hemangioblastoma, (c) PCC, (d) RCC, (e) or multiple renal and pancreatic cysts; or (4) a pathogenic VHL variant. This evidence concerns the gene VHL and von Hippel-Lindau disease.