INTRODUCTION: Uterine sarcomas are rare forms of malignant neoplasm, comprising about 3% of all malignant uterine tumors, representing less than 1% of all gynecologic malignancies. Low cure rates often occur due mainly to distant metastases, usually to the lungs. Aggressiveness, high rates of local recurrence, distant metastasis and poor prognosis with overall two-year survival less than 50% are common features of uterine sarcomas. Despite the low prevalence, these tumors are of great interest because of their multiple morphological and clinical features. OBJECTIVE: This article will be focused on the uterine sarcomas general aspects, etiology, prognosis, treatment and molecular features. METHOD: This review was performed using the Pubmed database to search for published articles. RESULTS: Little is known about the etiology of uterine sarcomas. Some studies have demonstrated the association between genetic events involving mutations in genes of the cell cycle and apoptosis and epigenetic in gynecologic sarcomas. Previous studies showed that chromosomal translocations have been identified, resulting in fusion genes that are constitutive and might involve the activation of transcription factors. Advances in molecular techniques have improved the diagnostic possibilities and allowed an improved understanding of the various pathologies. CONCLUSIONS: There are several factors that make the study of sarcomas a challenging issue, since those tumors are rare and the cell origin of each histologic type is nor well known Thus, molecular study of the events involved in the development of different types of cancer may lead to new strategies used in the diagnosis and treatment of these tumors.