Chronic granulomatous disease is characterized by recurrent life-threatening infections and the formation of granulomatous lesions caused by the failure of phagocytic oxidative microcidal activity that has resulted from a defect of the oxidase system. The fungal osteomyelitis of the spine in this disease is almost always caused by Aspergillus, and its treatment is not yet established although surgical management, antifungal agents, granulocyte transfusions or combination methods have been reported. We report a patient with aspergillosis who had a long-standing pulmonary lesion which subsequently spread to the adjacent thoracic wall and spine. After decompression and drainage by costotransversectomy, the severe pain was relieved but the kyphosis increased and a paraplegia developed slowly because of resistance to the antifungal therapy and the inability to perform a bone graft. The patient died 2 months after the operation because of pulmonary complications.