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Primary hepatoid adenocarcinoma of the endometrium is a rare tumour that is similar to hepatocellular carcinoma histologically. Here, a patient with primary hepatoid carcinoma of the endometrium with a high alphafetoprotein (AFP) level (90,508 ng/mL) is presented in a 75-year-old woman. The pelvic computed tomography and magnetic resonance imaging suggested a submucosal leiomyoma with degeneration or endometrial hyperplasia. However, the endometrial biopsy revealed a primary hepatoid carcinoma of the endometrium. The patient underwent a staging laparotomy including a total abdominal hysterectomy, bilateral salpingo-oophorectomy and lymph node sampling with pelvic cytology. The AFP level can be highly elevated in hepatoid carcinoma of the endometrium.