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Eosinophilic granulomatosis with polyangiitis is a systemic vasculitis. It could affect respiratory system, kidney, and central nervous system frequently; however, all body organs could be involved. Asthma and eosinophilic pneumonia are predominant manifestations in respiratory system. Bronchoalveolar lavage or lung biopsy may be used for diagnosis, but endobronchial lesion is not considered as a manifestation of eosinophilic granulomatosis with polyangiitis. Here we present a case of eosinophilic granulomatosis with polyangiitis with unusual endobronchial lesion which was confirmed by endobronchial biopsy.