The article describes the clinical signs of neurofibromatosis type I, in particular its bone manifestations. Also information on current approaches to diagnosis and surgical treatment of patients with neurofibromatosis type I and pseudarthrosis of the tibia is provided. Comprehensive treatment requires collaboration of experts of various disciplines taking into account that the lesion in neurofibromatosis is polysystemic, multiorgan and multidirectional. The choice of each stage of conservative and surgical treatment to restore the function of the limb should be differentiated. Long rehabilitation period provides social adaptation with proper improvement in the quality of life. Clinical case demonstrates professional use by the authors of treatment strategy resulting in a positive outcome — union of both bones of the tibia and partial recovery of limb function.