Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a rare autosomal dominant disorder that results from a germline mutation in the fumarate hydratase (<i>FH</i>) gene; it manifests as cutaneous leiomyomas, uterine fibroids, and renal cell cancer (RCC). The gene discussed is FH; the disease is renal cell adenocarcinoma.