Patients with von Hippel‐Lindau (VHL) have germline alterations in VHL and are at risk for benign and malignant lesions in the kidney, adrenal, pancreas, the central nervous system, retina, endolymphatic sac of the ear, epididymis, and broad ligament (Lonser et al., 2003) Clear cell renal cell carcinoma (ccRCC) arising in VHL patients exhibit loss of function of VHL, usually through loss of the wild‐type allele. Here, VHL is linked to clear cell renal carcinoma.