Uterine leiomyosarcoma (uLMS) is a rare and aggressive cancer, usually diagnosed incidentally at the time of myomectomy or hysterectomy. There have been concerns for several years about the fact that the inadvertent disruption of occult uLMS may have a negative impact on patient outcome. This study reviews the outcome and management of patients with a diagnosis of uLMS after surgery for presumed benign disease. We conducted a literature search in which 47 published English-language articles were obtained for evaluation. A total of 23 studies with outcomes data were included. It is evidenced that patients who underwent surgery with tumor disruption resulted in poorer outcomes compared with en bloc tumor, especially by power morcellation. The power morcellation was associated with an increased risk of recurrence, shorten time to recurrence, and upstage after re-exploration. Early re-exploration and surgical staging are appreciated for better prognosis and may alter postoperative treatment. We also updated on the incidence and preoperative evaluation to assess the risk of patient and give an effective counseling.