Abstract: Endometrial carcinoma is the most common parenteral tumor in female patients with Lynch syndrome. The disease is different from sporadic endometrial carcinoma, which is autosomal dominant genetic disease, and the pathogenesis and mechanism are the abnormalities of mismatch repair genes（MLH1，MSH2，MSH6 and PMS2）. Patients are much younger compared to sporadic endometrial carcinoma and pathological type is more diversity, including endometrial carcinoma, clear cell carcinoma, serous carcinoma and undifferentiated carcinoma and sarcoma, etc. Due to the higher risk of tumor recurrence and its great harmfulness of the disease，timely treatment of the Lynch syndrome related endometrial carcinoma is the key to inhibit the secondary tumor. This article is to summarize the new progress the etiology and pathogenesis of endometrial carcinoma，the clinical pathological characteristics and diagnosis，treatment and screening of Lynch syndrome related endometrial carcinoma.

Key words: Colorectal neoplasms, hereditary nonpolyposis, Endometrial neoplasms, Carcinoma, Diagnosis, Therapy