We report a rare case of secondary AA amyloidosis associated with chronic progressive pulmonary aspergillosis (CPPA) in which we could observe the correlative conditions of the two diseases. A 35-year-old man had a treatment history of pulmonary nontuberculous mycobacteriosis (NTM) and CPPA six years earlier. He was suffering from diarrhea when treating eruption of CPPA one year later. He was diagnosed as having AA amyloidosis by histopathological findings of the colon. Diarrhea disappeared and the histopathological findings improved as we treated CPPA. He had severe diarrhea again five years later when treating CPPA re-exacerbation. The colon biopsy again showed AA amyloidosis. Despite the treatment with intravenous and transbronchial infusion of amphotericin B after intubation, he died of heart and lung failure. This case suggests we should consider about the secondary AA amyloidosis when seeing patients with aspergillus infection and the importance of treatment of the infection itself.