Diffuse panbronchiolitis (DPB) is a chronic progressive obstructive airway disease. Clinically, DPB is manifested by productive cough and dyspnea and commonly combined with chronic sinusitis. Despite that the etiology of DPB is unknown, macrolide treatment is usually effective. A case of 45 year-old Taiwanese patient female who had DPB was reported by us. The patient's symptoms of cough and dyspnea were controlled within four months after the initiation of macrolides treatment of clarithromucin. Marked improvements on pulmonary function testing, chest radiography and high resolution computed tomography were also apparent. It may be distinguished between DPB and traditional chronic obstructive pulmonary disease by its rapid progression and the associated development of bronchiectasis and respiratory failure. It can be suggested that macrolide of clarithromycin is an effective therapy for DPB.