Uterine sarcomas comprise a heterogeneous group of diseases with different pathology appearance, clinical course and natural history. They account for only 3% of all uterine malignancies. The rarity of this entity has precluded the development of large and well designed randomised clinical trials, and for this reason the current management of some aspects of this disease is based on trials or retrospective studies with a low level of evidence. For this reason, it is mandatory to develop international cooperation to carry out clinically relevant clinical trials in this field. Accordingly, based on the relative rarity of these tumours, management of these patients should be centralised and must be performed by a multidisciplinary team including gynaecologic oncologist, pathologist, medical oncologist and radiation oncologist. This review focuses on the most accepted evidence about the management of uterine sarcomas. Although carcinosarcoma has been recently excluded from the sarcoma classification, some aspects of its treatment have also been included in this review.