Background Vascular rings are uncommon anomalies in which preferred strategies for diagnosis and management may vary among institutions. In this study, we reported our approach and a review of our 5-year experience. Methods From May 2006 to April 2011, 45 children (31 boys) with vascular rings underwent surgical repair at Beijing Children's Hospital. Nineteen patients (26%) had associated heart anomalies. Results There were two hospital deaths. At follow-up, 11 patients still had intermittent respiratory symptoms, but these symptoms had no effect on growth or physical activities. No patients required reoperation. Conclusions The rates of misdiagnosis and missed diagnosis of vascular rings are higher than those of other congenital heart diseases. A high index of clinical suspicion coupled with the use of computed tomography enables early diagnosis. Surgical repair can be performed successfully, although a number of patients will have persistent symptoms. Chin Med J 2012;125(8):1504-1507