Genetic studies have revealed several tumor suppressor genes [von hippel lindau (VHL), fumarate hydratase (FH), succinate dehydrogenase subunit B (SDHB) and folliculin (FLCN)] and proto-oncogenes (hepatocyte growth factor receptor MET) that are responsible for the development of renal cell carcinoma (RCC) [1]. The gene discussed is VHL; the disease is renal cell carcinoma.