In a mouse model of ccRCC induced by combined deletion of VHL, Trp53 and Rb1, the frequency of ciliation in tumor cells is significantly reduced [13], and loss of primary cilia was associated with onset of premalignant cysts and renal cancer [14–16], providing compelling evidence that the primary cilium functions as a tumor suppressor organelle in the kidney. Here, VHL is linked to nonpapillary renal cell carcinoma.