The Von-Hippel Lindau (VHL) disease, an autosomal dominant hereditary cancer syndrome caused by the loss-of-function germline mutation in the VHL gene [30], is characterized by the development of hemangioblastoma of the central nervous system and retina, ccRCC, and pheochromocytoma [31–32]. The gene discussed is VHL; the disease is nonpapillary renal cell carcinoma.