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, intraand 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%