Uterine sarcomas are an uncommon and heterogeneous group of tumors that account for 3-7% of the malignant neoplasms of the uterus and approximately 1% of all malignant tumors of the female genital system. The main clinical manifestations are abnormal uterine bleeding in pre- or postmenopausal women. Pelvic pain, abdominal distension, urinary urgency, and profuse and fetid leukorrhea are other frequent complaints. The authors present a case of a 48-year-old patient that was in amenorrhea for 2 years, who sought treatment for vaginal bleeding. On physical examination, the abdomen was distended, painful in the hypogastrium and upon examination of the external genitalia, it was observed the exteriorization of an amorphous “mass”. The patient was submitted to uterine curettage. The results of the histological examination revealed leiomyosarcoma. Staging workup showed an enlarged uterus with endometrial lesion, and non-calcified pulmonary nodules. The patient underwent a pan-hysterectomy and chemotherapy, and is under oncologic treatment. The authors call attention for the unusual form of presentation of this entity.