A number of monogenic autosomal-dominant hereditary tumor syndromes, such as Lynch, Li-Fraumeni, and melanoma-astrocytoma syndrome, caused by heterozygous pathogenic germline variants in cancer predisposition genes (CPGs), such as PMS2, TP53, and CDKN2A, are associated with an increased risk of gliomas [34, 37]. Here, PMS2 is linked to glioma.