The BAP1 tumor predisposition syndrome (BAP1-TPDS) has been characterized and associated with an increased risk for BAP1-inactivated melanocytic tumors (formerly known as atypical Spitz tumors), renal cell carcinoma, basal cell carcinoma, hepatocellular carcinoma, cholangiocarcinoma, meningioma as well as a number of other tumors suspected but not yet confirmed to be related to BAP1-TPDS [68]. This evidence concerns the gene BAP1 and cholangiocarcinoma.