Antineutrophil cytoplasmic antibody (ANCA) negative pulmonary limited-form granulomatous with polyangiitis (GPA) is a rare type of GPA. A 53-year-old female had been followed as the possible pulmonary infarction of bilateral lungs for 4 years without any therapy. Chest computed tomography(CT) examination of the patient showed newly appeared nodular lesions in the lungs, which were suspected as malignancy by positron emission tomography (PET) -CT. Thoracoscopic lung biopsy of the lesions was performed and histopathological diagnosis was GPA showing multiple granulomas with vasculitis. Since both C and P-ANCA were negative and no evidence of kidney involvement, we finally diagnosed the lung lesions as ANCA negative limited-form GPA.