Recent advances in next-generation sequencing (NGS) and their implementation in large cohort of cancer patients (e.g., The Cancer Genome Atlas (TCGA) projects), have led to the identification of additional genes frequently mutated in ccRCC, such as PBRM1 (≈40–50%), SETD2 (12%), BAP1 (10%), and KDM5C (5%) [9,10,11]. The gene discussed is BAP1; the disease is nonpapillary renal cell carcinoma.