A wide variety of neoplasms of varying histogenesis occur within the ovary. We report the first case of a primary ovarian myoepithelioma, a diagnosis made on the basis of the morphologic features coupled with immunoreactivity with epithelial and myoid markers. The tumor had a lobulated appearance with variable architectural patterns including anastomosing cords, trabeculae, and nests of epithelioid to spindled tumor cells within a hyalinised and focally myxoid stroma. Fluorescence in situ hybridization for EWS gene rearrangement and reverse transcriptase polymerase chain reaction for EWSR1-POU5F1 and EWSR1-PBX1, molecular abnormalities which are found in some extrasalivary myoepitheliomas, were negative. In reporting this unique neoplasm, we discuss the wide differential diagnosis generated by the case.