Mutations in the VHL gene can cause Von Hippel–Lindau syndrome (VHLS), which is a dominantly inherited familial cancer syndrome predisposing to a variety of malignant and benign neoplasms, most frequently retinal, cerebellar, and spinal hemangioblastoma, renal cell carcinoma, pheochromocytoma, and pancreatic tumors. This evidence concerns the gene VHL and renal cell carcinoma.