Uterine tumor resembling ovarian sex cord tumors (UTROSCTs) are uncommon neoplasms that histologically resemble ovarian sex cord tumor. Over 50 cases have been reported up to recently. Clement and Scully reported 14 cases of UTROSCT, which they devided into two groups by clinicopathological features. Group I tumors are endometrial stromal sarcomas with partially sex-cord like elements (less than 40% of total tumor volume). On the other hand, group II tumors are mostly or entirely composed of sex-cord like elements. We have experienced a very rare case of type II UTROSCT at the uterine fundus in a 52-year-old woman and report it with a brief review of the literatures.