A highlight has been given to the role of germline monoallelic variants in cancer predisposition in genes involved in the recognition and repair of DNA damage, such as the ATM, PALB2, and Fanconi anemia genes (Thompson et al., 2005; Renwick et al., 2006; Antoniou et al., 2014; Helgason et al., 2015; Rebbeck et al., 2015; Esteban-Jurado et al., 2016; Esai Selvan et al., 2019). The gene discussed is ATM; the disease is cancer.