Von Hippel–Lindau (VHL) disease is a hereditary cancer syndrome caused by mutations of the VHL gene resulting in different tumour subtypes including haemangioblastoma (HB) of the retina and the nervous system, clear cell renal cell carcinoma (ccRCC) and pheochromocytoma and paraganglioma (PPGL)5. This evidence concerns the gene VHL and nonpapillary renal cell carcinoma.