Patients with uterine leiomyosarcoma (LMS) typically present with vaginal bleeding, pain, and a pelvic mass, with atypical presentations of hypercalcemia and eosinophilia also being reported.Radiographic evaluation with combined positron emission tomography/computed tomography may assist in diagnosis and surveillance in women with uterine LMS, which is commonly used with stage and tumour grade as prognostic indicators and a recently developed riskassessment index to predict disease-specific survival.Recent studies have shown that the addition of adjuvant therapy after surgical management does not seem to improve survival, and ovarian preservation does not appear to negatively impact outcome.