Primary pulmonary amyloidosis is a rare disorder that appears in three forms: tracheobronchial, nodular parenchymal, and diffuse parenchymal. We report the case of a 46-year-old women with extensive tracheobronchial amyloidosis which presented with a 2-year history of dyspnea and with signs of severe fixed obstruction in pulmonary function tests. Computed tomography of the thorax demonstrated marked thickening of the trachea and the central bronchial tree with substantial narrowing of the main, lobar, and segmental bronchi. Transbronchial specimen showed typical birefringence under polarizing microscope after staining with Congo Red. We did not find hints for systemic amyloidosis.