The primary tracheo-bronchial amyloidosis is a rare entity with long lasting and progressive course. Precise diagnosis can be established on the basis of pathological features seen in samples derived from the airways, obtained during fibreoptic bronchoscopy or during lung biopsy sometimes. Describing the case of an insidious course of primary tracheobronchial AL type amyloidoisis, that was initially recognised and treated as a chronic obstructive pulmonary disease. The authors represent its clinical course and the diagnostic difficulties. The changes in the respiratory tract, both radiological and endoscopic suggested a tuberculous or proliferative process. They were responsible for a severe increasing dyspnoea, due to bronchial obstruction, with muco-haemoptic expectoration. A forceps resection of the endobronchial lesions enabled to established the right diagnosis and further treatment limited to local procedures.