Abstract: Objective To describe the clinical features and outcome of Wegener′s granulomatosis in children. Methods A retrospective study of 10 patients diagnosed as Wegener′s granulomatosis was conducted at Department of Pediatrics, Peking Union Medical College Hospital from October 1990 to July 2010 to analyze the clinical manifestations, examination results, pathological data, treatment and outcome. Results Of 10 patients, 6 were males and 4 were females. The ages of patients at disease onset varied from 7 to 17.1 years. The median age was 13.9 years. Time for diagnosis ranged from 2 to 24 months and follow-up time ranged from 4 months to 19 years. ①Seven children presented with fever at disease onset, fatigue in 3 patients and weight loss in 2 patients. Upper airway involvement occurred in all patients. Eight patients had pulmonary involvement. Renal involvement occurred in 4 patients. Joints, skin, eyes and nervous system were rarely involved. ②c-ANCA positive reaction was seen in 8 patients. ③Sinus imaging studies were taken in 9 patients, sinusitis in 4 patients, sinus mass in 3 patients and violation of eyes in 2 patients. Eight patients underwent chest CT examination, multiple pulmonary nodules with or without cavity formation in 5 patients, invasive lesions in 2 patients, and pleural effusion in 1 patient. Brain MRI showed ischemic changes in 2 patients. ④Pathological examination (7 nasal mucosa samples, 2 lung samples, 1 kidney sample) showed necrotizing granuloma and (or) vasculitis. ⑤All children were given corticosteroids and cyclophosphamide at the beginning of treatment. Treatment was changed to use glucocorticoid and methotrexate in 2 patients and 1 patient was given cyclosporine A. All patients were remitted after treatment. ⑥Seven patients relapsed, 1 patient had renal insufficiency and 1 patient died of respiratory failure during the period of follow-up. Conclusions The upper respiratory tract, lung and kidney were the most commonly involved organs in Wegener′s granulomatosis. Positive c-ANCA and pathological examination were helpful to the diagnosis. Treatment with corticosteroid and immunosuppressive could achieve good outcome.