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Osteogenesis imperfecta (OI) is characterized by bone fragility and is often accompanied by spinal deformity. Surgical treatment for early-onset scoliosis in patients with OI is hazardous and difficult due to the bone fragility and rigidity of the deformity. A case of early-onset scoliosis with OI that was treated using growing-rod surgery is presented. The patient was an 11-year-old girl with type 4 OI. At the age of 4 years, she was noted to have scoliosis. Preoperative radiographs showed that the Cobb angle, thoracic kyphosis angle, a