A 67-year-old woman presented with postmenopausal vaginal bleeding. Full body imaging demonstrated an intrauterine mass with deep myometrial invasion but no nodal or other metastatic disease. Uterine curettage was performed. Histologically, the tumor was an endometrioid adenocarcinoma with sarcomatous element and a hepatoid component, the latter was immunohistochemically positive for alpha-fetoprotein, HepPar-1, and arginase-1. The patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. Serum alpha-fetoprotein level decreased from 31896 ug/l preoperatively to 2063 ug/l postoperatively. Eight weeks later, a rise in serum alpha-fetoprotein was detected, and a biopsy-proven vaginal recurrence was diagnosed. Palliative chemotherapy led to tumor shrinkage and a concurrent decrease in the serum alpha-fetoprotein level. A rise in serum alpha-fetoprotein, refractory to second-line chemotherapy, was accompanied by subsequent development of ureteric obstruction, ascites, and radiological evidence of peritoneal metastases. This is an unusual case of uterine carcinosarcoma with an alpha-fetoprotein-producing hepatoid adenocarcinoma component. Serum alpha-fetoprotein level corresponds to disease recurrence and progression.