While mutations in Von-Hippel Lindau (VHL) gene have long been implicated in the pathogenesis of ccRCC [1,2], the Cancer Genome Atlas (TCGA) helped identify additional causative genes, including those in the chromosome 3p region adjacent to VHL, such as polybromo-1 (PBRM1); BRCA associated protein 1 (BAP1); and SET domain containing 2 (SETD2) [28]. The gene discussed is SETD2; the disease is nonpapillary renal cell carcinoma.