A number of hereditary RCC syndromes have been identified (12), such as Von Hippel-Lindau (VHL) disease, Birt-Hogg-Dubé (BHD) syndrome, Hereditary Leiomyomatosis and Renal Cell Carcinoma (HLRCC), and Hereditary Papillary RCC (HPRC), which is caused by germline mutations in VHL, BHD, FH and MET, respectively. The gene discussed is FH; the disease is hereditary leiomyomatosis and renal cell cancer.