Abstract: Lynch syndrome (LS) is the most common hereditary syndrome that predisposes patients to colorectal cancer. Lynch syndrome is defined as a hereditary predisposition to colorectal as a result of a germline mismatch repair (MMR) gene mutation. LS is the most common performance of hereditary nonpolyposis colorectal cancer. Many researches find that MMR gene mutations also cause endometrial carcinoma. While, our knowledge of LS associated with endometrial carcinoma is very limited, some opinions are still controversial. This review focuses on clinical pathological advances in LS associated with endometrial carcinoma and significance of screening in endometrial carcinoma.