Abstract
Primitive neuroectodermal tumors are high-grade malignant neoplasms. These are uncommon entities for the female genital tract. The treatment, management and follow-up period of Ewing's tumors are not well-defined because of their rarity in the genital tract. Surgical debulking is the mainstay treatment in all cases. After debulking surgery, patients receive chemotherapy and/or radiotherapy and there is a relation between disease stage and survival. Herein, we present a case of ovarian primitive neuroectodermal tumor with a review of previously reported cases.