Background: Neurofibromatosis type 1 is a hereditary disease that can lead to pseudoarthrosis of various long bones. Rarely, pseudoarthrosis affects the forearm, and to the best of our knowledge, this is the first reported case of pseudarthrosis of the distal radius treated by this unique method. Methods: We present a case of a 7-year-old male who underwent multiple operations to treat pseudoarthrosis of the distal radius. Results: After failed conservative and operative fixation augmented with autologous iliac crest bone grafting, the patient underwent a successful double-barrel vascularized free fibula graft while preserving the physis of the distal radius. Conclusion: Double-barrel vascularized free fibula bone graft can be successfully used as the definitive treatment in refractory cases of distal forearm pseudarthrosis and we believe that our technique can be applied to all cases of pseudarthrosis in other patients with a similar presentation and lesion location.