The aim of this study is to describe the outcome of surgical treatment for pediatric patients with forced vital capacity (FVC) <40% and severe vertebral deformity. Few studies have examined surgical treatment in these patients, who are considered to be at a high risk because of their pulmonary disease, and in whom preoperative tracheostomy is sometimes recommended. Inclusion criteria include FVC <40%, age <19 years and diagnosis of scoliosis. The retrospective study of 24 patients with severe restrictive lung disease, who underwent spinal surgery. Variables studied were age and gender, pre- and postoperative spirometry (FVC, FEV1, FEV1/FVC), preoperative, postoperative and late use of non-invasive ventilation (BiPAP) or mechanical ventilation, associated multidisciplinary treatment, type and location of the curve, pre- and postoperative curve values, type of vertebral fusion, intra- and postoperative complications, duration of intensive care unit (ICU) stay and length of postoperative hospitalization. Mean age was 13 years (9-19) of which 13 were males and 11 females. Mean follow-up was 32 months (24-45). The etiology was neuromuscular in 17 patients and other etiologies in 7 patients. Mean preoperative FVC was 26% (13-39%). Eight patients had preoperative home BiPAP, 15 preoperative in-hospital BiPAP, and 2 preoperative mechanical ventilation. Nine patients had preoperative nutritional support. Preoperative curve value of the deformity was 88 degrees (40 degrees -129 degrees ). Nineteen patients with posterior fusion alone and 5 with anterior and posterior fusion were found. Mean duration of ICU stay was 5 days (1-21). Total postoperative hospital stay was 17 days (7-33). Ventilatory support in the immediate postoperative includes 16 patients requiring BiPAP and 2 volumetric ventilation. None of the patients required a tracheostomy. The intraoperative complications include one death due to acute heart failure; immediate postoperative, four respiratory failures (2 required ICU readmission) and one respiratory infection; and other minor complications occurred in six patients. Overall, 58% of patients had complications. Percentage of angle correction was 56%. After a follow-up of 30 months, FVC was 29% (13-50%). In conclusion, corrective scoliosis surgery in pediatric patients with severe restrictive lung disease is well tolerated, but the management of this population requires extensive experience with the vertebral surgery involved, and a multidisciplinary approach that includes pulmonologists, nutritionists and anesthesiologists. Currently, there is no indication for routine preoperative tracheostomy.