SummaryThe authors report an observation of Wegener’s granulomatosis with pulmonary, bronchial, renal, cutaneous and sinusal involvement. Five years ago, the patient was referred to us because of bilateral diffuse pulmonary infiltrates of unknown origin. A cortico therapy was introduced at that time and a complete clearance of the pulmonary infiltrates was noted. Nineteen months after the treatment’s withdrawal, the disease recurs with the reappearance of pulmonary infiltrates. Beyond these unusual clinical aspects, histological examination of the bronchial biopsies were of diagnostic value.