To evaluate functional, clinical and radiological outcome achieved by intramedullary nailing and bone grafting in case of congenital psudoarthrosis of tibia. Introduction: Congenital pseudoarthrosis of the tibia is a rare disease with reported incidence of approximately 1 in 250000 live births. It is characterized by segmental osseous weakness resulting in deformities of the bone and spontaneous fractures which progresses to a tibial non – union. The etiology is unknown. The goals of surgery are to restore the tibial alignment, achieve bone union and re-establish the length of the tibia. Materials and Methods: We present a case of a 24 year old female with congenital pseudoarthrosis of left distal third of tibia. Patient was treated with excision of the pseudoarthrosis followed by reduction and fixation using intramedullary expert tibia nail and bone grafting from contralateral ASIS. Radiograph showed union of the tibia at three months follow up. Patient had limb length discrepancy of 3 cm and was ambulating with a shoe raised. The patient was satisfied with her current functional status and had adapted well.