Von Hippel–Lindau (VHL) disease (OMIM 193300) is a hereditary kidney cancer syndrome that is an autosomal dominant disease caused by mutations of VHL gene, characterized by multiorgan and multicenter tumors, such as renal cell carcinoma (RCC), pancreatic cyst or tumor (PCT), central nervous system hemangioblastoma (CHB), retinal angiomas (RA), pheochromocytoma (PHEO), papillary cystadenoma of the broad ligament or epididymis, and endolymphatic sac tumor (Ong et al., 2007). This evidence concerns the gene VHL and renal cell carcinoma.