A 16-year-old Japanese boy was admitted to our institution in September 2000 because no apparent callus had appeared around a fracture after 6 weeks of cast fixation. Physical examination revealed a tenderness of the right lower leg, and multiple small subcutaneous tumors and café-au-lait spots in extremities and trunk. Radiographs showed the fracture of the right lower tibia with bony sclerosis and a localized fusiform osteolytic lesion at the fracture site. The affected tibia bowed anteriorly and the medullary space in the lower tibia was narrow. A diagnosis of Boyd type IV congenital pseudoarthrosis of the tibia was made. Treatment with low-intensity pulsed ultrasound (US) stimulation (LIPUS) was administered for 20 min/day, and a nonweight-bearing gait was continued with a cast or brace fixation. At 6 months after the treatment, a small amount of bridging callus was seen. We continued the treatment for 1 year until the solid fusion was observed on radiographs and the patient started full-weight-bearing.