Recently, it has been estimated that 2–8% of RCC cases are of hereditary origin with identified germline pathogenic or likely pathogenic variants (GPV) in genes including BAP1, FH, FLCN, MET, MITF, PTEN, SDH genes (SDHA, SDHAF2, SDHB, SDHC, SDHD), TSC1, TSC2, VHL, and WT1 [17–22]. The gene discussed is MITF; the disease is renal cell carcinoma.