This report described a 2-year-old boy who presented with severe respiratory distress and stridor. Bronchoscopy and CT revealed a mass in the left anterolateral tracheal wall and histopathology showed a tracheal inflammatory myofibroblastic tumor. Initial removal by rigid bronchoscopy resulted in prompt recurrence of the tumor. Therefore he underwent tracheal surgical resection. A bronchoscopy at 12 months after surgery did not show any recurrence sign.