Primary site is ∼80% pleural, ∼10% peritoneal and ∼10% pericardial or testicular.1, 2, 3 Pleural mesothelioma (PM) is known to be closely associated with asbestos exposure, with chronic inflammation being the main driver of carcinogenesis.4, 5, 6, 7 In addition, heterozygous germline alterations in BAP1 leading to BAP1 tumor predisposition syndrome can cause Mendelian distribution of mesothelioma occurrence across generations.8 Here, BAP1 is linked to BAP1-related tumor predisposition syndrome.