Background and Objectives: Vascular rings refer to a group of anomalies of the great arteries that cause respiratory symptoms or feeding problems. The purpose of this study was to analyze a series of patients with vascular rings in a single center. Methods: Retrospective review of all patients identified as having vascular rings between 1997 and 2010 in the Seoul National University Children���s Hospital. Results: Thirty-five patients were reviewed and categorized as to specific arch anomaly. Median age at diagnosis was 7 month-old (range: birth to 8.7 year-old). The average follow-up duration was 5.73짹5.01 years. Twenty-eight (80%) patients were confirmed as vascular rings by cardiac CT or MRI. The types of vascular rings are double aortic arch in 9 (25.7%), right aortic arch with persistent left ligament arteriosum in 7 (20%), right aortic arch with aberrant right or left subclavian artery in 8 (22.9%), others in 3 (8.6%) and pulmonary sling in 8 (22.9%). Eight (22.9%) patients were asymptomatic and presenting symptoms of other patients were gastrointestinal system in 14 (40%) patients and respiratory system in 16 (45.7%) patients. Associated cardiovascular anomalies were present in 15 (42.9%) of 35 patients. Four patients were identified as CATCH 22 syndrome. The compressed lesions were esophagus in 19 (54.3%) patients and airway in 21 (60%) patients. Operative treatments were done in 20 (57.1%) patients with definite symptoms: double aortic arch in 7/9 (77.8%), right aortic arch with persistent left ligament arteriosum in 3/7 (42.9%), right aortic arch with aberrant right or left subclavian artery in 3/8 (37.5%) and pulmonary sling in 6/8 (75%). The median interval between diagnosis and operation was 6 days (range: 0 to 8 months). Four patients died even though mortality from vascular ring itself occurred in only one patient with pulmonary sling with severe trachea stenosis. Two patients underwent tracheostomy. Conclusions: Vascular rings include several types of anomalies with different presenting symptoms and prognoses. Overall prognosis of vascular ring itself was good in the most patients. Early diagnosis and timely surgical treatment in the symptomatic patients are mandatory and medical management with close follow-up are recommended for patients with mild symptoms.