Granulomatous polyangiitis (GPA) is a multiple systemic necrotizing vasculitis. Diagnosis of pulmonary nodules in GPA is still challenging in clinical practice, however, other extrapulmonary manifestations, serology, and histopathology may help the diagnosis of GPA. This case series was of limed GPA with one of the largest pulmonary nodules which had a poor treatment response in contrast with previous literature.