Uterine tumors resembling ovarian sex cord tumors (UTROSCTs) are very rare uterine neoplasms. They mostly behave in a benign fashion but cases were reported with extra-uterine spread. There are less than 50 cases reported in the literature so far according to the present authors' knowledge. We report a case of 45-year-old woman with UTROSCT concurrent with adenomyosis and complex hyperplasia with atypia of endometrium and her three-year follow up.