Uterine sarcomas account for less than 1% of gynecological malignancies and 2–5% of all uterine malignancies. Such sarcomas mainly include leiomyosarcoma (LMS) and endometrial stromal sarcoma (ESS). Additionally, inflammatory myofibroblastic tumor (IMT) and endometrial carcinoma arising in adenomyosis can occur as uterine myometrial tumors. Their differentiation from leiomyoma (LM), particularly degenerated LM and the malignant tumors, is challenging, but preoperative diagnosis is very important for the patient’s management. We demonstrate the useful and compulsory findings to differentiate between uterine myometrial malignant tumors and degenerated LM with an unusual appearance.