This report describes a case of negative pressure pulmonary edema (NPPE) after orthognathic surgery for osteogenesis imperfecta (OI). An 18-year-old male with a prognathic mandible, earlier diagnosed with OI Type I, was referred by his orthodontist. Presurgical laboratory tests showed his bleeding time and coagulation to be normal, and his chest radiograph was unremarkable. We performed Le Fort I osteotomy and bilateral vertical ramus osteotomy. Continuous bleeding was noted during the operation. After extubation, the patient became agitated and made vigorous inspiratory efforts. His oxygen saturation dropped to 40%, and he was reintubated atraumatically. NPPE was suspected. The patient was transferred to the intensive care unit. Although OI has been linked to several risk factors during and after surgery, such as decreased platelet function causing abnormal bleeding and low-ventilation due to chest wall deformity, follow up periods summing to 5 years showed good bone healing, good occlusion and no further complaints. We predict that prolonged operation time and increased bleeding volume may have influenced the onset of NPPE. Reports of orthognathic surgery for OI and NPPE after orthognathic surgery are currently limited. Our results suggest that a minimally invasive surgery is preferable for OI patients. (c) 2017 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.*