Dysfunctions in the skeletal cells caused by mutations in the neurofibromatosis type 1 (NF1) gene can result in osteopenia/ osteoporosis and impaired bone healing in patients with NF1. We report a case of NF1 with tibial nonunion successfully treated by reamed intramedullary nailing, nonvascularized fibular bone grafting, and demineralized bone matrix application. The entity of neurofibromatosis and treatment of tibial nonunion are briefly reviewed.