Uterine leiomyosarcoma (LMS) are rare but aggressive tumours with poor clinical outcomes regardless of stage. Most tumours are identified by histopathology at time of surgery, and pre-operative diagnosis remains a clinical challenge. Management of early-stage LMS relies on surgical resection. Cytotoxic chemotherapy remains the mainstay of therapy for advanced-stage, recurrent or metastatic LMS, and includes single or combination doxorubicin-, ifosfamide- or gemcitabine-based regimens. Recent interest in genetic biomarkers led to developments of targeted therapies for LMS, although more research is needed to understand the molecular complexities underlying LMS to guide the development of novel treatment strategies.