Given the observations of single pathogenic variants predisposing to multiple tumour types arising in distinct tissues, such as with BAP1 (uveal melanoma, mesothelioma, meningioma, clear cell renal cell carcinoma and cholangiocarcinoma [58, 59]), BRCA1 or BRCA2 (breast, ovarian, uveal melanoma) [60–62], it is plausible that other examples exist that have diverse effects that have yet to be described. The gene discussed is BRCA2; the disease is cholangiocarcinoma.