Uterine tumors resembling ovarian sex cord tumors (UTROSCT) are rare, usually benign, polypoid or nodular neoplasms which generally arise in the fourth to sixth decade of life. We report a case of a 74-year-old woman who presented with vaginal bleeding and remarkable uterine enlargement. Abdominal hysterectomy with bilateral salpingo-oophorectomy was performed and a diagnosis of UTROSCT was made. Immunohistochemistry is mandatory for a correct diagnosis and a panel of at least two markers of sex cord differentiation is recommended. Differential diagnoses include leiomyosarcoma, UTROSCT and ESTSCLE, mixed müllerian tumor and metastatic ovarian sex cord tumor.