Pleomorphic rhabdomyosarcoma of the uterus is a rare malignant tumor. It is connected with postmenopausal abnormal vaginal bleeding and abdominal pain. We report a case of a 66-year-old postmenopausal woman diagnosed with abnormal vaginal bleeding and abdominal pain. Vaginal ultrasonography showed enlarged uterus, 82 mm x 64 mm in size. The patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy with postoperative chemotherapy due to pleomorphic rhabdomyosarcoma of the uterus. The patient died 2.5 years after the surgery as a result of a rapid spread of the neoplastic process. The case of rhabdomyosarcoma, together with the review of the literature, is presented in the following work. We find that the rarity of this histological entity makes it particularly worthy of publication.