BACKGROUND: Vascular rings are rare congenital malformations that lead to various degrees of respiratory difficulties or feeding problems by forming a complete or partial ring that compresses the trachea, the esophagus, or both. CASE PRESENTATION: We describe the case of an 18-month-old boy with recurrent respiratory symptoms and dysphagia. Chest radiograph revealed slightly hyperinflated lung fields bilaterally. Barium esophagography suggested vascular ring anomaly. CT angiogram revealed a vascular ring, consisting of a double aortic arch, compressing the trachea and esophagus. The echocardiogram revealed did not reveal any associated congenital heart defect. He underwent a successful surgical repair. After a follow-up period of one year, the patient was free of respiratory problems and pulmonary infections, and without recurrence of dysphagia. CLINICAL DISCUSSION: The clinical manifestations of vascular rings are heterogeneous. Vascular rings may be either asymptomatic or more frequently, may present with respiratory and/or gastrointestinal symptoms that depend on the degree of tightness of the ring and subsequent tracheoesophageal compression. CONCLUSION: A high index of suspicion is needed to make an early diagnosis so that timely treatment options may be offered to prevent prolonged and unnecessary treatments and life-threatening complications.