Since the isolation of the von Hippel-Lindau gene (VHL) in 1993, Bi-allelic VHL loss, either through mutation, hypermethylation, or chromosomal loss, has been widely reported in most ccRCC patients, indicating the pivotal role of VHL in the genetic pathogenesis of ccRCC [15]. Here, VHL is linked to nonpapillary renal cell carcinoma.