A 46-year-old man afflicted with recurring infection and bone fracture consulted our hospital because of general malaise and increase of sputum. He was given a diagnosis of chronic necrotizing pulmonary aspergillosis, and underwent right lower lobectomy. Six months later, chronic necrotizing pulmonary aspergillosis become exacerbated. Slightly improvement was obtained with voriconazole. Two months later, this disease become reactivated, and slightly improved with itraconazole and amphotericin B. Subsequently, hyper-IgE syndrome was diagnosed in him by pathognomonic face, recurring infection and bone fracture, chronic necrotizing pulmonary aspergillosis, elevated IgE, and eosinophilia. We suggested that the pathogenic cause of chronic necrotizing pulmonary aspergillosis in this case was hyper-IgE syndrome. After that, chronic necrotizing pulmonary aspergillosis was reexacerbated. We added micafungin, increased itraconazole, interferon gamma, and so on. As a result, his chest radiograph and symptoms improved slowly. Cases of hyper-IgE syndrome are rare.