Most patients with Wegener's granulomatosis initially present with upper airway symptoms, persistent rhinosinusitis or otitis media. We report the case of a young female who presented with progressive dyspnea, inspiratory stridor and barking cough due to a subglottic stenosis. We would like to stress this often unrecognized type of presentation. The differential diagnosis, the limitations of histopathological examinations, and the interest of looking for anticytoplasmic antibodies in the diagnosis of Wegener's granulomatosis, are briefly discussed.