Vascular rings are a relatively rare type of congenital cardiovascular malformation, accounting for about 1% to 2% of congenital heart disease, often with respiratory tract as the main clinical manifestation, and are easily misdiagnosed or missed. The vascular ring is not self-healing, and the clinical symptoms gradually worsen with age, requiring surgical treatment. Double aortic arch is the most common type of vascular ring, followed by pulmonary artery sling. The clinical manifestations of vascular rings are mainly symptoms of respiratory obstruction, such as wheezing, shortness of breath, cough, respiratory distress, and recurrent respiratory infections, which are easily misdiagnosed clinically as asthma and upper respiratory tract infection. Some children may present with slow feeding, recurrent vomiting and feeding difficulties due to esophageal compression, which can easily be diagnosed as congenital esophageal stricture. The disease needs to be thought of in children who present clinically with chronic wheezing, shortness of breath, dyspnea and/or dysphagia and slow feeding to avoid delay in diagnosis and treatment. The diagnosis of vascular rings is not difficult. Spiral CT or MRI can accurately diagnose and localize vascular lesions, and bronchoscopy should be performed in cases of severe or long-segment tracheal stenosis. In symptomatic cases of vascular rings, surgical treatment should be performed as soon as possible once the diagnosis is confirmed. Even if there are no symptoms at the time of diagnosis, surgical treatment is necessary because of the risk of softening of the compressed trachea over time. Because of airway obstruction, preoperative lung infection in children is often not easily cured, and surgery should not be postponed in those with lung infection; surgical release of airway compression helps control lung infection. Surgical treatment includes dissection of the vascular ring and complete release of the tissue surrounding the ring. Surgery can be performed through a lateral thoracic incision, except for pulmonary artery sling or combined with other intracardiac malformations which are best operated through a median incision. The management of combined tracheal stenosis is the key to the surgical treatment of vascular rings and directly affects the final outcome of the surgery. Localized tracheal stenosis is better improved by surgical release of the vascular ring compression, while surgery for long segments of tracheal stenosis requires staging. The respiratory functional status of the child needs to be followed up for a long time.