Recent advances in genetics and pathology have improved our understanding of diagnosis and staging of uterine sarcomas. The major types of uterine sarcomas include leiomyosarcoma, low-grade endometrial stromal sarcoma, undifferentiated endometrial sarcoma, adenosarcoma and carcinosarcoma. The distinctive biological behavior and poor overall survival of uterine sarcoma create challenges in the management of these tumors. We herein present a comprehensive review of taxonomy, epidemiology, pathology, imaging findings and natural history of a wide spectrum of uterine sarcomas.