Germline P/LP variants were identified in ATM, ATR, BAP1, CHEK2, DDX41, FANCM, HAX1, MRE11A, MSH6, MUTYH, NF1, SAMD9L, and TMEM127.<h4>Conclusions and relevance</h4>In this case series of 161 patients with mesothelioma, 16% had confirmed germline P/LP variants. The gene discussed is ATR; the disease is mesothelioma.