Primitive neuroectodermal tumors (PNET) of the ovary are rare tumors with an exclusive or almost exclusive malignant neuroectodermal composition, and are generally regarded as a monodermal expression of an ovarian teratoma. The tumors are basically identical with the lesions of the same name occuring typically in the central nervous system of children. These tumors consist chiefly of undifferentiated small cells resembling neuroblasts. There are also mature, well- differentiated neuroectodermal cells, such as astrocytes and ependymal cells. We report a case of ovarian PNET with glial and neuroblastic differentiation and focal teratomatous foci of non-neural tissue in a 17-year-old female.