Posterior mediastinal tumors of neurogenic origin commonly arise from the sympathetic or intercostal nerves. However, anterior mediastinal tumors rarely originate from the vagus nerve, and primary neurogenic tumors of the trachea are extremely uncommon. A 19-year-old man was admitted to an emergency department in sudden acute respiratory distress. A tracheostomy was performed and he was transferred to our Ear Nose and Throat Department for further investigation. A bronchoscopic biopsy was taken of a mass occupying the tracheal lumen and intraoperative frozen section examination suggested a schwannoma, so tracheal resection was performed. Although rare, primary tracheal schwannoma should be considered in the differential diagnosis of sudden respiratory distress of unknown origin.