We report two cases of "uterine leiomyoma with tubules" as a new pathological entity. Since these are biphasic neoplasms (composed by epithelial and mesenchimal elements), the differential diagnosis is between mixed mullerian tumors and uterine tumors resembling ovarian sex cord tumors (UTROSCTs). In the differential diagnosis, the mixed mullerian tumors are easily excluded because of histological and immunohistochemical features. UTROSCTs are similar to the lesions we reported, and the differential diagnosis requires positivity for some immunohistochemical markers as inhibin, CD99, calretinin, Melan-A. Our conclusions are that to perform a diagnosis of UTROSCT at least two immunohistochemical marker have to be expressed; in the present case they didn't, so we call the lesion "leiomyoma with tubules".