Background: Data on endovascular therapy (EVT) in patients with stroke attributable to CeAD is scarce. We performed a systematic review and meta-analysis of all studies comparing EVT to intravenous thrombolysis (IVT) in CeAD-patients and compared EVT to IVT with regard to 3-month outcome and complications. Additionally, we included data of IVT-/EVT-treated CeAD-patients from a Swiss multicenter IVT-/EVT registry based cohort study. Methods: We systematically searched the pubmed® database to identify all existing studies comparing IVT to EVT in CeAD-patients. Studies were eligible if IVT-treated CeAD- patients were compared to EVT-treated patients, and 3-month outcomes were reported as modified Rankin Scale (mRS) score. Outcome measures in this study were favorable 3-month outcome (i.e. mRS 0-2), excellent 3-month outcome (i.e. mRS 0-1), symptomatic intracranial hemorrhage (sICH) and death. Additionally, in a Swiss multicenter IVT-/EVT registry based cohort-study (SWISS) we identified all consecutive patients with ischemic stroke attributable to CeAD and included outcome data of these patients in our analyses. In a meta-analysis across all studies, we compared EVT to IVT with regard to primary and secondary outcome measures using a fixed-effect Mantel-Haenszel model. Results: The literature search yielded 388 results. We identified 7 eligible studies for a systematic comparison of EVT versus IVT. Data on 62 patients from the SWISS cohort were included as an additional study. In total, we compared EVT (n=102) versus IVT (n=110) in 212 CeAD-patients. With regard to the occurrence of mRS 0-2, there was no significant difference between both treatment groups (OR 1.04 (95% CI 0.57-1.88)). Separate Data on mRS 0-1 was available in 5 studies (OR 0.88 (95% CI 0.39-2.00)). There was no difference between groups with regard to death at 3 months (OR 0.66 (0.22-1.94)) which was separately reported in 6 studies. For sICH there were too few data (n=5 patients) available for meta-analysis. Conclusion: In this systematic review and meta-analysis of all existing studies comparing EVT to IVT in CeAD patients there was no clear signal of superiority of EVT. Further investigation of EVT with up-to-date devices is warranted.