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Background: Adenoid cystic carcinoma (ACC) of the ovary is an extremely rare malignancy referred to eight cases in the literature.Here we report a new case of adenoid cystic carcinoma tumor of the ovary (Mullerian carcinoma with myoepithelial differentiation, adenoid cystic carcinoma-like). Case: The patient is 54-years old female with stage IIIC ovarian cancer treated with cytoreductive surgery and platinum-based adjuvant chemotherapy. During the subsequent follow-up period, the patient was diagnosed with a recurrence of the ACC of the ovary in the pelvis. She was treated with second-line palliative chemotherapy, including Carboplatin and Caelyx, a total of six cycles. The patient is alive 44 months since diagnosis. The prognosticators of survival are based on the previous 8 cases. The disease stage is the most crucial prognosticator for survival; other relevant factors to a worse outcome in ACC of the ovary are advanced age, residual tumor after initial surgery, and suboptimal cytoreduction. Conclusion:The best treatment currently is unknown; however, optimal cytoreduction surgery and platinum-based chemotherapy appear to be effective for the ACC of the ovary. Despite this tumor's intrusive nature, the prognosis can be improved if diagnosed early and treated.