The prognosis of Wegener's granulomatosis (WG) improved following the introduction of immunosuppressant agents. We, however, still experience some difficult cases that are resistant to treatment. Early diagnosis and treatment may improve the prognosis of WG. We reviewed patients with WG attending the Department of Otorhinolaryngology, Kitasato University Hospital. Of the 13 patients reviewed, 5 are still alive. A definite diagnosis was difficult to make without the presence of certain characteristic features. Recently, cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) has gained importance in the diagnosis of WG and five of our patients were positive for c-ANCA. Close long-term follow-up of WG is recommended: remission was observed in two of our patients and side-effects of the treatment were identified in four.