BackgroundCongenital pseudarthrosis of the tibia (CPT) is a challenging problem in orthopedic practice, with high rates of non-union, refracture, and residual deformities. After union, long-term follow-up is required to manage late post-union complications. This study aimed to assess the outcomes of the Ilizarov technique in the management of CPT.Materials and methodsThis retrospective study included patients with CPT treated with the Ilizarov method between 2005 and 2018. Intramedullary rods were used in 9 cases and iliac bone graft was used in 12 cases. An orthosis was applied till the end of follow-up in all cases. The American Orthopaedic Foot and Ankle Society (AOFAS) scale was used for the evaluation of the functional outcomes.ResultsThis study included 16 patients, 11 males and 5 females, with an average age of 5.4 ± 2.8 years. Seven cases had multiple previous surgeries. Six patients had neurofibromatosis. The mean follow-up period was 5.8 ± 3.4 years. The average AOFAS score improved significantly from 47.5 ± 7.6 preoperatively to 78.9 ± 8.9 at the latest follow-up.Union was achieved in 15 cases, and persistent non-union occurred in one case. The clinical results were excellent in one patient, good in seven cases, fair in 6, and poor in 2 cases. The radiological results were excellent in one patient, good in seven cases, fair in seven, and poor in one case.ConclusionsThe Ilizarov technique combined with intramedullary rod and primary or secondary bone graft provides a high union rate of CPT and can achieve simultaneous effective management of problems related to pseudarthrosis, including non-union, deformity, limb shortening, and adjacent joint contracture and subluxation.Level of evidence Level IV.