The renal cell carcinoma is recognized by an extensive energy metabolism reprogramming, which is related to mutations in specific genes, including VHL, MET, BAP1, TFE3, TFEB, FLCN, MITF, FH, SDHB, SDHC, SDHD, TSC1, TSC2, PBRM1, SETD2 and KDM5C. The gene discussed is FH; the disease is hereditary clear cell renal cell carcinoma.