Heterozygous germline mutations in FH followed by the loss of the second wild-type allele (loss of heterozygosity-LOH) predispose to hereditary leiomyomatosis and renal cell cancer (HLRCC), a syndrome characterised by benign cutaneous and uterine leiomyomata, renal cysts, and type 2 papillary renal tumours [8, 16]. The gene discussed is FH; the disease is hereditary leiomyomatosis and renal cell cancer.