Germline mutations that inactivate the VHL tumor-suppressor gene cause von Hippel–Lindau disease, which predisposes individuals to the most common form of kidney cancer, clear-cell renal cell carcinoma (ccRCC), as well as various other tumors such as hemangioblastomas and paragangliomas (1, 2). Here, VHL is linked to nonpapillary renal cell carcinoma.