The authors report a case of Wegener's granulomatosis, whose initial manifestation was an isolated endobronchial stenosis. In connection with this observation, it calls to mind, within the scope of the pulmonary granulomas (in view of such a rare clinical presentation) the difficulties of differential diagnosis, in particular with bronchocentric granulomatosis, and stresses the need for pulmonary histological examination to open lung biopsy.