HLRCC is an autosomal dominant condition characterized by germline mutations in the fumarate hydratase (FH) gene, and susceptible individuals are at risk for developing cutaneous and uterine leiomyomas, as well as an aggressive form of type 2 papillary renal cell carcinoma [17,18]. The gene discussed is FH; the disease is hereditary leiomyomatosis and renal cell cancer.