Clinical features and treatment outcomes of 16 patients with Wegener's granulomatosis were evaluated retrospectively. The ages of the patients ranged from 28 to 81 years with a median age of 53 years. There were 7 males and 9 females. Thirteen patients had a nasal necrotic granuloma, 5 patients had a history of otitis media with hearing loss, 4 patients had an orbital invasion, and 2 patients had hypertrophic pachymeningitis. 12 patients had a localized type of Wegener's granulomatosis, the other 4 patients had lung lesions, and 2 patients had kidney lesions. The laboratory results of 7 patients were positive for C-ANCA, 7 patients had typical pathologic appearance in the nasal mucous membrane, but the other had no typical pathologic signs. These patients were diagnosed with Wegener's granulomatosis based on the clinical, histological and laboratory findings, and predonisolone was administered to all patients. We should pay attention to diagnosis of Wegener's granulomatosis, especially with a localized type of Wegener's granulomatosis with otolaryngological symptoms alone, without positive laboratory results for C-ANCA and typical pathologic appearance.