Although molecular genetic studies have shown that mutations of several genes are associated with the pathogenesis of ccRCC, including von Hippel-Lindau (VHL), set domain-containing 2 (SETD2), BRCA1-associated protein-1 (BAP1), polybromo-1 (PBRM1), and lysine-specific histone demethylase 5C (KDM5C) [5–8], additional genes are being identified to be related to RCC from cancer genomic studies, which may have prognostic, predictive and therapeutic relevance [9]. The gene discussed is PBRM1; the disease is nonpapillary renal cell carcinoma.