The understanding of the pathogenesis of the commonest histological type, clear cell renal cell carcinoma (cc RCC), has been considerably advanced by the recognition that the gene encoding the von Hippel-Lindau (VHL) protein is defective in patients with VHL disease and in the majority of sporadic cc RCCs resulting in activation of a hypoxic pattern of gene expression [2,3]. Here, VHL is linked to renal cell carcinoma.