Wegener's granulomatosis is an rare disease of unknown etiology but generally considered a hypersensitivity disorder. This disease was first described by Klinger in 1931 and noted as a distinct clinicopathological syndrome by Wegener in 1936, as rhinogenic granulomatosis ; necrotizing granuloma of upper or lower respiratory tract, generalized focal necrotizing angitis and a focal necrotizing glomerulitis. This disease is classified based on anatomic site of involvement ; classic form of Wegener's granulomatosis and limited from of Wegener's granulomatosis which is absent of renal invovement. The treatment of choice is cyclophosphamide therapy with or without corticosteroid in this disease. The authors, recently, have observed 1 case of classic form and 3 cases of limited form of Wegener's granulomatosis and report these with literature review.