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Dedifferentiated endometrioid adenocarcinoma (DEAC) consisted of a combination of undifferentiated and differentiated carcinoma is more aggressive than other conventional endometrioid adenocarcinomas.A 33-year-old woman with atypical vaginal bleeding was refereed to our hospital. She had an endometrial biopsy in a local clinic which showed differentiated endometrioid carcinoma with trophoblastic components. High levels of β-Human chorionic gonadotropin (β-hCG) and alfa-fetoprotein (AFP) were detected in the patient's serum.The patient underwent total hysterectomy with bilateral salpingo-oophorectomy, total omentectomy and systemic pelvic lymphadenectomy in our center.Pathological investigation indicated that the tumor had well differentiated and undifferentiated adenocarcinoma as well as trophoblastic components.Serum β-hCG and AFP dropped significantly after operation. But three weeks later, the patient had developed pulmonary metastases and elevation of serum β-hCG. She died of the disease five months after surgery.DEAC with trophoblastic differentiation seems to follow an aggressive course with early metastasis and poor clinical prognosis. However, due to small number of cases, further studies are necessary.