Inflammatory myofibroblastic tumors (IMTs) are rare childhood neoplasms, with benign clinical course. Although etiology of IMTs are not clear, recent studies have reported that IMT is a true neoplasm rather than a reactive or inflammatory lesion. IMTs are rarely seen in adults and tracheal involvement is also rare both in adults and also in children. We describe a 16-year old female patient who was misdiagnosed and treated as asthma in another center for a few months and presented with acute respiratory distress due to upper airway obstruction. Computerized tomography (CT) of the chest and rigid bronchoscopy revealed a mass lesion that was nearly totally obliterating tracheal lumen. Bronchoscopic resection was performed under general anesthesia and the final pathological diagnosis was tracheal IMT. (C) 2014 The Authors. Published by Elsevier Ltd.