Respiratory tract involvement with amyloid is rare. We retrospectively reviewed the records of 10 patients with biopsy-proven pulmonary amyloidosis who were evaluated at our hospital between 1996 and 2003. All relevant information, such as clinical, historical, laboratory, radiographic, and histologic data were examined. We had 5 cases of tracheobronchial amyloidosis, 4 cases of nodular parenchymal amyloidosis, and 1 case of diffuse parenchymal amyloidosis. Four cases were primary and 6 cases were reactive amyloidosis that accompanied other diseases, such as rheumatoid arthritis, Sjögren syndrome, and so on. Tracheobronchial amyloidosis and diffuse parenchymal amyloidosis had symptoms similar to those caused by various airway disorders, such as cough, hemoptysis, and shortness of breath. However, the nodular parenchymal amyloidosis had no symptoms. Nine cases were diagnosed by only bronchoscopic examination and 1 case was diagnosed by thoracoscopic lung biopsy.