Congenital pseudarthrosis of long bones occurs commonly in the tibia and also in the radius, ulna and clavicle. Neurofibromatosis or skin manifestations occur in up to 50% of patients with pseudarthrosis. The defect may be noted at birth but is usually observed later in early childhood. In most cases, there is a pathological fracture, bone pain and deformity. The cortex is thin and the medulla wide. An area of radiolucency is seen at the site of pseudarthrosis. There is progressive tapering of the bone ends towards the site of pseudarthrosis.