Lynch syndrome (LS) is caused by germline pathogenic variants (PVs) in one of the DNA mismatch repair (MMR) genes (MLH1, MSH2, MSH6, or PMS2) or by deletions in the epithelial cell adhesion molecule gene (EPCAM), which increases susceptibility to colorectal and endometrial cancers and other tumors (1–3). Here, MSH6 is linked to endometrial cancer.