VHL and neoplasm: Most cases are sporadic, and about 20% to 38% of patients also have Von-Hippel-Lindau (VHL) disease, which is an autosomal dominant genetic condition with an incidence of 1/27,300 to 1/45,000.[1–3] The VHL gene is located on chromosome 3p25–26 and is an important tumor suppressor gene that contains three exons.[4,5] The VHL gene is transcribed to a 4.5-kb-long mRNA that encodes a VHL protein (PVHL) containing 213 amino acids.