Fumarate hydratase–deficient renal cell carcinoma (FH-RCC) is a rare subtype of kidney cancer, characterized by either somatic or germline aberration of fumarate hydratase (FH) gene, in which pathogenic germline mutation of FH gene is associated with hereditary leiomyomatosis renal cell carcinoma (HLRCC) syndrome [1, 2]. The gene discussed is FH; the disease is hereditary clear cell renal cell carcinoma.