Heterologous sarcomas of the uterus are rare neoplasms. We report a rare case of heterologous uterine sarcoma composed of leiomyosarcomatous and liposarcomatous components with a brief review of literature. A 53-year-old woman had vaginal spotting. The endometrial biopsy performed at a local clinic revealed a high grade spindle cell sarcoma. Pelvis magnetic resonance imaging showed about 5.4 cm sized multiseptated T1 and T2 high signal mass in a uterine fundus without lymph node metastasis or pelvic wall involvement. Total laparoscopic hysterectomy, bilateral salpingo-oophorectomy, pelvic and para-aortic lymph node dissection, and peritoneal washing cytology were performed. Pathologic diagnosis was made as leiomyosarcoma with liposarcomatous differentiation (lipoleiomyosarcoma). The patient received adjuvant postoperative radiotherapy (5040 cGy). Follow up images showed no evidence of disease 8 months after surgery.