Vascular rings are rare anomalies accounting for 1% to 3% of all congenital heart diseases with a similar frequency in both sexes, which can result in a variable degree of extrinsic compression of the trachea and esophagus. We report the case of a 46-year-old woman with symptomatic dyspnea and double aortic arch that caused esophageal tracheal compression. Considering the little impact of the functional vascular anomaly in our case, a conservative approach was adopted with clinical and functional annual follow-up.