The diagnostic and therapeutic management of uterine leiomyosarcomas (uLMS) represents a real challenge. Any failure to diagnose or suspect the disease will result in inappropriate treatment and damage to the patient’s health, with a major impact on patient survival. The major difficulty lies in the rarity of uterine sarcomas compared with the frequency of benign pathologies, in particular, uterine leiomyomas. In the case of broad ligament leiomyosarcomas, they are often mistaken for adnexal pathologies, resulting in inappropriate management, with no margin, or even tumour rupture. The diagnostic approach is key to improving practices, with monitoring of imaging interpretation algorithms, percutaneous biopsies in patients of childbearing age, pathologic specimens analysed by experts, combined with an evaluation of the genomic index. Management and discussion at a multidisciplinary meeting specialising in sarcoma improves the quality of the surgery, avoiding inappropriate surgery, indication of adjuvant treatment and patient survival. It ensures that treatment is in line with international standards of care. New molecular biology tools will probably make it possible to identify patients who would benefit from adjuvant chemotherapy. New chemotherapy molecule is improving survival in metastatic leiomyosarcoma, despite the prognosis remains poor.