Uterine tumor resembling sex cord stromal tumor (UTROSCT) is an unusual and very rare group of tumors, with approximately 70 cases reported to date. The tumor was first described in 1976, and has since been characterized using immunochemistry and cytogenetics. A pathologic diagnosis of UTROSCT is made when the tumor shows characteristic histological features and, in addition, positive immunohistochemical staining of markers of sex cord differentiation (calretinin, melan-A, CD56, CD99 or inhibin). Imaging features, however, are non-specific and cannot be used to reliably differentiate this tumor from other uterine masses, such as leiomyomas. UTROSCT appears heterogeneous on ultrasound, and demonstrates hyperenhancement on earlier arterial phase on computed tomography. These tumors appear T1 hypointense or intermediate and T2 intermediate, but more often T2 hyperintense relative to myometrium; it may have slightly more restricted diffusion as a possible differentiating imaging feature. Due to the rare possibility for metastasis, the present authors believe surgical excision is a reasonable course of management.