Congenital pseudoarthrosis of the tibia (CPT) is one of the most difficult orthopedic problems to treat, often resulting in a deformed leg or a juvenile amputee. The condition commonly presents as a "prefracture stage" in infancy, showing anterolateral tibial bowing, medullary canal sclerosis, diaphyseal constriction, or cystic lesions. This pre-fracture stage does not invariably lead to fracture, but once fracture does occur, pseudoarthrosis formation routinely follows. Various morphological classification systems have been proposed for congenital pseudoarthrosis tibia. Numerous types of bone graft techniques, mostly autologous, have been advocated. The Ilizarov technique (in use since 1971) is a comprehensive approach to all aspects of CPT. This technique simultaneously attends to various aspects of the condition, including deformity, shortening defects, infections, articular function, weight-bearing and valgus ankle. (22) The aim of this essay will be to review the literature regarding the management of this problem.