FH mutations have been implicated in the pathogenesis of various diseases, most typically hereditary leiomyomatosis and renal cell carcinoma (HLRCC), a cancer syndrome characterized by cutaneous uterine leiomyomas and papillary type II renal cell carcinomas (RCC), one of the most aggressive forms of renal carcinoma. Here, FH is linked to hereditary leiomyomatosis and renal cell cancer.