Uterine lipoma (UL) is a rare tumor frequently presenting as leiomyolipoma. Even more uncommon is the association of UL and endometrial carcinoma (EC) for which only two cases have been reported. We present the case of a 73 years old female of French origin complaining of vaginal bleeding. Initial examination under general anesthesia found a 13 cm length uterine cavity with a large tumor located in the anterior wall. After curettage, the histopathologic analysis diagnosed a moderately differentiated adenocarcinoma of the endometrium. Patient work-up showed no evidence of extension outside the uterus (stage Ib). Treatment consisted of pelvic and iliac lymph node external irradiation with subsequent vaginal intracavitary irradiation followed, 6 weeks later, by total extra-fascial hysterectomy without lymph node dissection. Pathologic examination found a small EC with limited infiltration of the myometrium (stage Ia) associated with a 10 x 9 x 7 cm yellowish fibrolipoma type tumor without smooth muscle pattern. First described by Lobstein in 1816, UL are uncommon and casually diagnosed. The association with EC is rare and without evident relationship. Ultrasound (US) and computed tomographic (CT) appearances allow a diagnostic approach. US show a highly echogenic central tumor with a thin moderately echoic rim. CT appearance of UL is a central, well delimited fat-density tumor. The etiopathogenesis of UL is unclear, attributed to a possible leiomyoma evolution for Willen or a myometrial cell metaplasia for Brandfass.