Proximal location of congenital pseudarthrosis of the tibia (CPT) is uncommon, and its management challenging, risking to end in amputation. We here report a case of proximal CPT managed in a limb-sparing perspective and followed up until the end of growth. A 17-year-old girl presented with type-1 neurofibromatosis and proximal CPT. Initial X-ray showed severe pseudarthrosis of the tibia with bone atrophy, 12-cm shortening and femorotibial and femoropatellar dislocation. Inter-tibiofibular graft and fibular tibialization were performed. At end of follow-up (age 33 years), fusion had been obtained. For orthoprosthetic and cosmetic reasons, a Boyd amputation of the tarsus was performed when the patient was 22 years of age. The functional result was very good, with 0–100° knee mobility. CPT, when proximal, completely disorganizes the knee joint, which is otherwise usually unaffected by this pathology. To achieve a good result, a limb-sparing treatment should combine correction of the tibial axis and of the dislocation of the knee, fibula osteosynthesis and bone graft.

Level IV retrospective