Uterine leiomyosarcomas are rare but challenging tumours. They occur most commonly around or shortly after the menopause. Their clinical behaviour is very variable, from highly aggressive to very indolent. Most are diagnosed unexpectedly and thus initially operated on by general gynaecologists. This article discusses the important surgical issues and the role of adjuvant treatments such as radiotherapy and chemotherapy. Important new international trials are opening to address these issues. Relapsed disease is usually incurable, but a subgroup of patients may benefit from repeated surgical procedures, hormones and ablative therapies. The choice of drugs for chemotherapy is discussed. New approaches with targeted agents have yet to establish themselves in treatment of leiomyosarcomas.