Abstract: Objective:To investigate the clinicopathological features of epithelioid trophoblastic tumor (ETT) of the uterus with emphasis on its differential diagnosis.Methods:The clinicopathological data of 1 case of ETT was analyzed and the literature was reviewed.Results:The patient was a 30-year-old female with the symptoms of delayed menstruation and abnormal vaginal bleeding for half a year.Gross examination of the hysterectomy specimen revealed a limit-unclear lesion involving the left uterine wall.The tumor was 5 cm in size and was necrotic with areas of hemorrhage;microscopically,the tumor was composed of one type intermediate trophoblast cells arranged in nests and cords,which distributed in a geographical pattern with hyalinization or eosinophilic debris of tumor cell necrosis.The medium-sized tumor cells contained abundant eosinophilic or clear cytoplasm and distinct cell borders,round or oval nuclei with distinct nucleoli.Immunohistochemically,the tumor cells were positive for AE1/AE3,p63 and Ki-67,and partially positive for hCG.Conclusions:ETT is a rare gestational trophoblastic tumor that has malignant biological behavior of lower grade.It should be differentiated from cervical squamous cell carcinoma and other trophoblastic tumors.