Since the von Hippel–Lindau (VHL) disease tumor suppressor gene VHL was identified in 1993 as the genetic basis for a rare disorder, the von Hippel–Lindau (VHL) syndrome (OMIM 193300) has been an autosomal dominant hereditary condition characterized by a predisposition to tumors [8]. The gene discussed is VHL; the disease is von Hippel-Lindau disease.