Hepatoid carcinoma of the ovary (HCO), a rare invasive malignant tumor composed mainly of epithelioid cells, presented with unilateral or bilateral ovarian masses and elevated serum α-fetoprotein (AFP), has been found mainly in post-menopausal women. We hereby report on the case of a 53-year-old Chinese woman who presented with abdominal distension and a lower abdominal mass with high serum levels of CA-125 and AFP. She was later diagnosed with bilateral HCO. After surgery and following chemotherapy, the patient had no recurrence of tumor or ascites. The hepatoid cells were positive for AFP, p53 and CK7 by immunohistochemistry. Her serum CA-125 and AFP levels had decreased significantly after surgery. Our results suggest that testing and monitoring of serum levels of AFP and CA-125 are considered as potential biomarkers in the diagnosis and progression of this malignancy, and that tissue immunohistochemical staining for AFP, p53 and CK7, plays an important role in distinguishing HCO from other ovarian tumors.