Abstract: Objective: To investigate the clinical and pathological features of Wegener granulomatosis. Methods: 36 patients with Wegener granulomatosis were retrospectively analyzed. Results: The median age at presentation was 38.5 years（ranging from 12-65 years）.The initial symptoms of the 36 cases were running a fever（47%）,coughing with or without sputum（42%）,and having a stuffy and running nose（36%）.Most patients had multiple systems and organs involvement.32 patients（89%） had upper respiratory tract involvement,28 patients（78%） had lung involvement,and 19 patients（53%） had kidney involvement.68% of the patients were cANCA positive.The pathologic features were necrotic granulomatosis and microvasculitis.Chest radiograph and CT scan of the lung showed infiltrates,nodules and cavity disease. Conclusion: The clinical manifestations of Wegener granulomatosis are quite complicated,including multiple systems and organs involvement.The upper and lower respiratory tract and kidney are the most commonly involved organs.ANCA testing,Chest CT scan and histological biopsy play important roles in early diagnosis.