OBJECTIVE To describe a case of a patient with an epithelioid trophoblastic tumor (ETT) and review the literature regarding this new type of uterine trophoblastic tumor which is being reported with increasing frequency.There have been only 42 cases described in the world literature.METHODS Routine sections of the tumor were prepared and stained with H&E. Using the SP method, immunohistochemical staining, for AE1/AE3,hPL, PLAP, and α-inhibin antigens was conducted.RESULTS The patient was a 34 years old female who had delivered 12 years previously. She presented with amenorrhea for three months and vaginal irregular bleeding for 2 months. Her serum hCG level was 2,240 IU/L. After diagnostic curettage, an ETT was identified, and total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAHBSO) performed.On microscopic examination it was found that the tumor was composed of chorionic-type intermediate- trophoblastic cells. The tumor cell nests were distributed in a geographical pattern. Some cells were filled with an eosinophilic hyalinized degenerative material. Study of the immunophenotype of the tumor showed that AE1/AE3, hPL, hCG, and α-inhibin were positively expressed.CONCLUSION This is the 4th case report of an ETT in China. The tumor was identified as a new type of trophoblastic tumor combined with a focal chorioepithelioid carcinoma, a condition that is extremely rare. It consists of chorionic-type intermediate-trophoblastic cells, and is considered to have a Iowgrade of malignancy. ETT should be differentiated from a placenta-site trophoblastic tumor, placenta-site nodule, chorioepithelioid carcinoma, and cervical squamous cell carcinoma.