Lynch syndrome is caused by genetic or structural variants or hypermethylation in DNA MMR genes MLH1, MSH2, MSH6, and PMS2 and confers an increased predisposition to colorectal and endometrial carcinomas,11, 12 with the lifetime cumulative risk of EC for women with Lynch syndrome being in the range of 40%–60%.13 Here, PMS2 is linked to endometrial carcinoma.