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Endodermal sinus tumor (EST) of the ovary is extremely rare and little information exists about therapy and the role of second-look laparotomy in the management of this entity. A case of EST of the ovary in a 21 year old woman is reported. She received conservative surgery and six courses of combination therapy consisting of Vincristine, Actinomycin D and Cyclophosphamide before second-look laparotomy. Due to progression of the disease second-line polychemotherapy with Vinblastine, Bleomycin and Cisplatin was administered. This new regimen reduced the alpha-fetoprotein to normal levels although the patient was not free of disease on second-look laparotomy. Precise guidelines for the management of this disease, especially in advanced stages, are still lacking.