Abstract Granulomatosis with polyangiitis (GPA), formerly called Wegener’s granulomatosis, is a systemic disease with autoimmune small vessel vasculitis. It affects the upper (sinuses and nose) and lower (lungs) respiratory tract. It frequently involves the kidneys, lungs, eyes, ears, throat, skin and other body organs. We described a case of 42 years old man who was initially diagnosed as a case of community acquired pneumonia on the basis of clinical and radiological findings. Finally he was diagnosed as a case of granulomatosis with polyangiitis (Wegener’s granulomatosis) as the nasal biopsy showed ulcer with granuloma and c-ANCA found positive. Keywords: Granulomatosis with polyangiitis (GPA),Vasculitis, Nasal Mass, Haemoptysis.

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