Pure nongestational choriocarcinoma of the ovary is an extremely rare tumor. Histologically, it has the same appearance as gestational choriocarcinoma metastatic to the ovaries. The majority of patients with this cancer are younger than 20 years. Isosexual precocity has been documented in about 50% of patients whose lesions arise prior to menarche. The presence of hCG can be useful in monitoring the patient during and after treatment. Because of its rarity, there are only a few series of reports in which chemotherapy was used for these nongestational choriocarcinomas, but there have been complete responses reported to the MAC regimen (methotrexate, actinomycin D, and cyclophosphamide). However, as the BEP or EP is active in gestational choriocarcinoma, these regimens are also a reasonable approach for the nongestational lesions. The prognosis for ovarian choriocarcinoma has been poor, with the majority of patients having metastatic disease to organ parenchyma at the time of initial diagnosis.