Risk factors for kidney cancer include obesity, smoking, hypertension and multiple autosomal dominantly inherited cancer predisposition syndromes including von Hippel–Lindau (VHL), Birt-Hogg-Dubé (BHD) syndrome, hereditary leiomyomatosis and renal cell cancer syndrome (HLRCC), PTEN hamartoma tumour syndrome, hereditary pRCC, BAP1 tumour predisposition syndrome, succinate dehydrogenase subunit genes (SDHB, SDHC and SDHD) and constitutional chromosome 3 translocations (2,5,6). Here, BAP1 is linked to hereditary leiomyomatosis and renal cell cancer.