Abstract Objective To review the current evidence on the effects of intra-abdominal morcellation on survival outcomes of patients affected by unexpected uterine leiomyosarcoma (ULMS) and to estimate the risk of recurrence in those patients. Methods PubMed (MEDLINE), Scopus, Embase, Web of Science databases as well as ClinicalTrails.gov, were searched for data evaluating the effects of intra-abdominal morcellation on survival outcomes of patients with undiagnosed ULMS. Studies were evaluated per the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) and the American College of Obstetricians and Gynecologists (ACOG) guidelines. Results Sixty manuscripts were screened, 11 (18%) were selected and four (7%) were included. Overall, 202 patients were included: 75 (37%) patients had morcellation of ULMS, while 127 (63%) patients had not. A meta-analysis of these studies showed that morcellation increased the overall (62% vs. 39%; OR: 3.16 (95% CI: 1.38, 7.26)) and intra-abdominal (39% vs. 9%; OR: 4.11 (95% CI: 1.92, 8.81)) recurrence rates as well as death rate (48% vs. 29%; OR: 2.42 (95% CI: 1.19, 4.92)). No between-group difference in cumulative extra-abdominal recurrence (OR: 0.34 (95% CI: 0.07, 1.59)) rate was observed. Conclusions Our data support a significant correlation between uterine morcellation and an increased risk of intra-abdominal recurrence in patients affected by unexpected ULMS. However, the limited data on this issue and the absence of high level of evidence suggest the need of further studies designed to estimate the risk to benefit ratio of morcellation in patients with uterine fibroids and undiagnosed ULMS.