Pulmonary mucormycosis is an uncommon opportunistic fungal infection associated with diabetes mellitus, leukemia, lymphoma and other debilitating diseases. The diagnosis is made by the pathology demonstration of typical hyphae within the lung tissue, and requires aggressive treatment including surgical and medical methods for reducing the overall mortality. In Korea, the association of pulmonary murcomycosis with Wegener's granulomatosis is rare. However, pulmonary mucormycosis with diabetes has been reported in several cases. Recently, we treated a case of pulmonary murcomycosis in 42-year-old woman with Wegener's granulomatosis, who had recurrent pneumothorax and a rapidly progressive lung abscess. Here we report this case and review the medical literature.