BACKGROUND Congenital distal tibiofibular diastasis is an extremely rare disorder. The main feature at presentation is a clubfoot deformity with leg length discrepancy (LLD) due to a short tibia. Limb lengthening and reconstruction techniques today can correct the deformity, ending with a plantigrade and functional limb. METHODS We report a patient with congenital tibiofibular diastasis, who was initially treated with series of casts and later with ankle arthrodesis, but the deformity and LLDleg length discrepancy remained. We applied an Ilizarov frame and initially corrected the varus and equinus deformity of the foot. Then, we corrected the LLD leg length discrepancy with limb lengthening with distraction osteogenesis. We achieved a plantigrade stable limb, with a small amount of length inequality. CONCLUSIONS Congenital distal tibiofibular diastasis can be managed with staged correction and lengthening with the Ilizarov method.