BACKGROUND: The treatment of congenital pseudarthrosis of the tibia remains difficult and controversial. The purpose of this study was to evaluate the long-term results of a technique consisting of excision of the pseudarthrosis, autologous bone-grafting, and insertion of a Williams intramedullary rod into the tibia. METHODS: Twenty-one consecutive patients with congenital pseudarthrosis of the tibia were managed with this technique between 1978 and 1999, and the results were retrospectively reviewed. The mean age of the patients at the time of the latest follow-up was 17.2 years (range, seven to twenty-five years), and the mean duration of postoperative follow-up was 14.2 years (range, three to twenty years). RESULTS: Initial consolidation occurred in eighteen of the twenty-one patients. Refracture occurred in twelve patients; five fractures healed with closed treatment, five healed after an additional surgical procedure, and two ultimately required amputation. Ten patients had an ankle valgus deformity after tibial union. Eleven patients had a residual limb-length discrepancy of >2 cm; six required a contralateral distal femoral and/or proximal tibial epiphyseodesis, two had a tibial lengthening, and one used a shoe-lift. Five patients had an amputation: two, because of a recalcitrant fracture; two, because of a limb-length discrepancy (6 and 9 cm); and one, because of a chronic lower-extremity deformity. CONCLUSIONS: This technique produced a satisfactory long-term functional outcome in sixteen of twenty-one patients and should be considered for the management of congenital pseudarthrosis of the tibia.