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Granulomatosis with polyangiitis (GPA) is a systemic vasculitis that is associated with antineutrophil cytoplasmic antibodies (c-ANCA). It classically presents with sinonasal, pulmonary and renal involvement. We are presenting a case of a 32-year-old male who presented with septal perforation, crusting and nasal obstruction. He had been operated on twice for sinonasal polyposis. Relevant investigations revealed that he was actually suffering from GPA. The patient was started on remission induction therapy. A combination of methotrexate and prednisolone was started with a 2-weekly follow-up. The patient had experienced his symptoms for 2 years before presentation. This case highlights the importance of correlating ENT and pulmonary symptoms to reach the correct diagnosis.