Granulomatosis with polyangiitis is a small vessel vasculitis and ANCA related.30-year female presented with complaints of rhinorrhea for 4 months. Xray chest showed doubtful Right midzone opacity. Coagulation profile and hemogram was unremarkable with raised ESR and Proteinuria on urine analysis. Sputum studies for PTB was negative. HRCT thorax showed solitary soft tissue mass lesion in Right lower lobe with spiculated margins suggestive of neoplasm. Biopsy of Lung mass was suggestive of tissue was consistent with granulomatosis with polyangiitis and C – ANCA was positive. She developed ear, eye symptoms with skin rashes later. Diagnosis of Granulomatosis with polyangiitis with sinusitis, mastoiditis, scleritis, vasculitic skin rash, kidney proteinuria was made and treated with Prednisolone, Azathioprine, Mycophenolate Mofetil following which patient improved and is on follow up.