Abstract: Lynch syndrome (LS), an autosomal dominant genetic disorder caused by mismatch repair gene defects, is characterized by the susceptibility to multi-system malignancies. LS associated endometrial cancer (LS-EC) is the most common extraintestinal sentinel cancer of LS. In the era of precision medicine, early screening of LS-EC is of great significance to preventing the occurrence of other LS related malignant tumors. This paper reviews the research progress on screening methods and prevention of LS-EC, in order to provide reference for precise diagnosis and prevention of LS-related tumors.