Objective: The present study aims to review cases of extremely rare primary ovarian tumors and thus, to evaluate the distribution of rare primary ovarian pathologies. Various aspects of the presentation, diagnosis, and treatment of these tumors are discussed. Materials and Methods: A retrospective review of women with final pathology of rare primary ovarian tumors, which were managed at the Gynecologic Oncology Department of Zekai Tahir Burak Women's Health Education and Research Hospital, from 1995 to 2010 was undertaken. Results: Among the 2,210 women treated during the study period, extremely rare ovarian tumors accounted for 1.62%(36/2210) of all cases. Conclusion: It is important to be aware of these rare entities in the pathological diagnosis of ovarian tumors. Intraoperative frozen examination should be performed because rare benign conditions that mimic malignancy may not require radical surgery. The rarity of these tumors renders basic scientific advances more challenging.