Objective To summarize the experiences of single stage repair of persistent fifth aortic arch associated with stenosis and interrupted aortic arch and other cardiac anomalies,and to improve surgical effect of the diseases． Methods From Jan．2000 to May 2008，five patients with persistent fifth aortic arch were operated in this hospita1，the age at operation was 1.8-108.0 months and body weight 3.7-31.0 kg．Three patients had chronic heart failure and respiratory infection repeatedly.All patients received single stage repair. Results There were two early hospital deaths，one patient’s parents gave up all the therapy because of cardiac insufficiency, pulmonary hypertension crisis and severe pulmonary infection; another one died of severe pulmonary hypertension crisis，the low cardiac outflow and left heart failure. Three patients were followed up, followup time was 55.67±48.64 months. The results were excellent,and one patient had been followed up for 8 years,the latest magnetic resonance imaging showed that diameter of the enlarged fifth aortic arch was 9.3 mm. Conclusion Persistent fifth aortic arch operation can achieve good exposure,less incisional wound and excellent recovery through midline sternotomy.Because of systemic hypertension and the affection of associated anomalies the operation should be performed as early as possible.