Tracheobronchial amyloidosis(TBA) is a rare disorder which refers to localized aberrant protein deposits within the airways. Because of the lack of characteristic presentation, long lasting and insidious course, patients with TBA are often misdiagnosed as other pulmonary diseases. At present bronchoscopic biopsy was the gold standard for the diagnosis of TBA. We present a case of TBA which was misdiagnosed as asthma due to asthma-like wheezing and the effectiveness of anti-asthmatic treatment. The definite diagnosis was confirmed by CT scan, bronchoscopic findings and Congo red staining of biopsy specimen. The literature involving the recent progres-sion of diagnosis and treatment of TBA that is reviewed.