<h3>Study Objective</h3> To quantify the efficacy of medical management of uterine arteriovenous malformation (AVM) and compare efficacy between different classes of medication. We also evaluated for factors associated with treatment success and pregnancy outcomes following medical management. <h3>Design</h3> Systematic review and metanalysis. <h3>Setting</h3> Not applicable. <h3>Patients or Participants</h3> Thirty-two studies representing 121 premenopausal women with medically treated uterine AVM were identified via database searches of MEDLINE, Embase, Web of Science and cited references. <h3>Interventions</h3> Medical treatment with progestins, gonadotropin-releasing hormone agonists (GnRH-a), methotrexate, combined hormonal contraception (CHC), uterotonics, danazol, or combination of the above. <h3>Measurements and Main Results</h3> The primary outcome of treatment success was defined as AVM resolution without subsequent procedural interventions. Secondary outcome was treatment complication (re-admission or transfusion). The unadjusted overall success rate of medical management was 88% (106/121), while individual agent success rates were progestins (82.5%), GnRH-a (89.3%), methotrexate (90.9%), CHC (42.8%), uterotonics (100%), and danazol (66.6%). After adjusting for clustering effects, success rates for progestin (82.5%, 95% CI 70.1% to 90.4%, <0.001), GnRH-a (89.3%, 95% CI 71.4% to 96.5%, p<0.001) and methotrexate (90.0%, 95% CI 55.8% to 98.8%, p=0.028) were significantly different from the null hypothesis of 50% success. In a pairwise comparisons, progestins (OR 6.29, 95% CI 1.19 to 33.17, p=0.030) and GnRH-a (OR 11.13, 95% CI 1.63 to 76.10, p=0.014) were more efficacious compared to CHC. The agents with the lowest adjusted proportion of complications were progestins (10.0%, 95% CI 3.3% to 26.8%, p<0.001) and GnRH-a (10.7%, 95% CI 3.5% to 28.4%, p<0.001). No clinical factors were found to predict treatment success. Twenty-six subsequent pregnancies are described, with no reported recurrences of AVM. <h3>Conclusion</h3> Primary medical management for symptomatic uterine AVM is a reasonable approach in a well selected patient. This data should be interpreted in the context of significant publication bias.