Type 2 VHL disease shows a high risk of pheochromocytoma (PCC) and germ line missense mutations is subdivided into high risk (2B), low risk (2A), or absence (2C) of Renal cell carcinoma (RCC) and heamangioblastoma is correlated with function of pVHL to impair HIF-1α activity [1, 2]. The gene discussed is VHL; the disease is renal cell carcinoma.