Smooth muscle tumors of the uterus represent a spectrum of diseases that range from benign leiomyoma to malignant leiomyosarcoma. The leiomyoma is the most common of these neoplasms. Clinically, it is important to fully understand the differences in clinical presentation, biologic behavior, and management for patients with benign leiomyoma, smooth muscle tumors of uncertain malignant potential, and leiomyosarcoma. The goal of this review is to present the most recent information about common smooth muscle tumors of the uterus including their etiology, histopathology, radiographic and clinical presentations, and available treatment options.