Based on the current nomenclature, autosomal dominant Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC, MIM150800) describes the exact cancer syndrome with germline heterozygous variant of the FH gene, which encodes fumarate hydratase, an enzyme in the Krebs cycle [4]. The gene discussed is FH; the disease is hereditary leiomyomatosis and renal cell cancer.