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To compare the current treatments for congenital pseudarthrosis of tibia, a total of 33 studies were reviewed. Vascularized fibular graft with external fixation or combined fixation had the fastest time till initial union (P<0.05). Bone morphogenetic protein had no advantage in terms of initial union, time till union, and refracture rates. This meta-analysis supports that fixation methods with corticocancellous bone autograft utilizing the combined technique of Ilizarov external fixation and intramedullary rod stabilization ensure a statistically significant reduction in the number of refractures compared with standalone fixation methods and would be the preferred method for preventing long-term complications in patients with congenital pseudarthrosis of tibia.