A broad tumor spectrum accompanies BAP1 pathogenic germline variants [10,11] and although this spectrum has not yet been fully elucidated, pathogenic variants in BAP1 is known to predispose to cutaneous and uveal melanoma and mesothelioma and is suspected of playing a role in the development of other cancers such as breast cancer, cholangiocarcinoma [12], cancer of the pancreas [10] and basal cell carcinoma [13,14]. The gene discussed is BAP1; the disease is cholangiocarcinoma.