Objective：To summarize the clinical features，diagnosis and treatment of congenital vascular ring in children. Methods：Clinical data of 60 patients with congenital vascular rings in Children’s Hospital of Chongqing Medical University from January 2003 to January 2013 were retrospectively studied. All children underwent computed tomography angiography（CTA），echocardiography，gastrointestinal barium meal，fiberoptic bronchoscopy. Results：Sixty cases including 40 males and 20 females，aged from 1 day to 6 years，were enrolled in our study. The most frequent diagnosis was pulmonary artery sling（25 cases，41.67%），followed by double aorta arch（21 cases，35%） and right aortic arch with left ductus arteriosus（12 cases，20%）. Twenty-eight cases（46.7%） combined with heart malformations. Forty five cases（46.7%） combined with tracheobronchial stenosis，10 cases combined with esophageal stricture.The diagnosis rate of CTA was 81.82% and that of echocardiography was 53.33%. 52 cases（87%） had a history of respiratory symptoms including difficulty in breathing，recurrent cough and wheezing，and recurrent respiratory tract infections. Forty patient who underwent surgery included left pulmonary artery transplantation（15 cases），double aortic arch devascularization（12 cases），artery catheter/ligament cut-off（12 cases），left subclavian artery occlusion（1 case）. Seven patients died. Vascular ring combined with complete tracheal ring was a risk factor for patients underwent surgery（P<0.05）. Patients underwent surgery remained respiratory symptoms for up to 6 months，however，they had significant improvement in the postoperative period. Conclusion：Children with recur－rent respiratory symptoms such as recurrent respiratory tract infections，chronic cough and wheeze，dysphagia should be examined for the presence of congenital vascular ring. Echocardiography combined with CTA is the best way to diagnose vascular ring. Early cor－rect diagnosis and timely surgical treatment are the keys to survival.