Discussed is a case of a stage IV pure endodermal sinus tumor (EST) of the ovary that was treated with a PVB protocol following cytoreductive surgery. The serum alpha-fetoprotein (AFP) dosage before treatment was 83,546 ng/ml, and a complete clinical response was achieved with the disappearance of pleural effusions, ascites, and normalization of the serum AFP. A second-look laparotomy was substituted by serial AFP determinations and the patient remained free of disease for two years. Recently, complete remissions and long term survivals of patients with a stage I-II disease have been reported, though a prolonged or a complete remission of a stage IV EST of the ovary is still a rarity.