We report a case of a 14-year girl with severe congenital scoliosis (CS) and type ІІ respiratory failure (RF), who underwent preoperative halo-gravity traction in combination with intraoperative orthopaedic surgery on the spine based on the results of physical examination, pulmonary function tests (PFTs), computed tomography (CT), and blood gas analysis. The patient's coronal and sagittal Cobb's angles changed from 100° to 45° and 40°, respectively, and RF changed from type ІІ to type І after treatment. Scoliosis was corrected well, and RF was improved. During follow-up for nearly 3 years, no loss of deformity correction and no serious complications occurred, and the patient showed a good clinical outcome and balanced spine. Key Words: Congenital scoliosis, Halo-gravity traction, Respiratory failure.