Primary ovarian undifferentiated non-small cell carcinoma, neuroendocrine type.

Background: Non-small cell neuroendocrine carcinoma (NSCNEC) of the ovary is a rare aggressive neoplasm, characteristically arising in association with a surface epithelial tumor.

Case: A 27-year-old woman with no significant past medical history presented with a 17-cm left ovarian tumor. She underwent an exploratory laparotomy with resection of the pelvic mass. A diagnosis of stage IA non-small cell neuroendocrine carcinoma was rendered. She was subsequently treated with Taxol and Carboplatinum.

Conclusion: This is a report of a rare primary ovarian non-small cell neuroendocrine carcinoma. In contrast to prior reports, this neoplasm was not associated with a surface epithelial tumor.