Congenital pseudarthrosis of the tibia is characterized by anterolateral deformity of the tibia and shortening of the limb. Its etiology remains unclear. Although several classification systems have been proposed, none provides specific guidelines for management. Treatment remains challenging. The goal is to obtain and maintain union while minimizing deformity. The basic biologic considerations with surgical intervention include resection of the pseudarthrosis and bridging of the defect with stable fixation. Intramedullary stabilization, free vascularized fibula, and Ilizarov external fixation are among the most frequently used methods of treatment. In addition, bone morphogenetic protein recently has shown promise. Nevertheless, despite improvements in healing rates with congenital pseudarthrosis of the tibia, the potential for amputation in failed cases persists.