Wegener's granulomatosis (WG) is an autoimmune disease which has a clinical predilection for the upper airways, lungs and kidneys. It is a necrotising granulomatous vasculitis which is associated with a distinct autoantibody--the antineutrophil cytoplasmic antibody (ANCA). A heightened index of suspicion by clinicians is needed in the diagnosis of this complex and rare condition. A multidisciplinary approach should then be used to treat this chronic multisystem disease. Treatment involves the use of various regimens of corticosteroids and immunosuppressive medication. Mortality due to WG has been significantly decreased by this therapy. In this article, we focus on clinical manifestations and review the salient histologic, laboratory and serologic features and treatment.