Double aortic arch (DAA) is a very rare congenital vascular anomaly, characterized by the encircling trachea and esophagus, resulting feeding and/or respiratory problems. In this article, a two-month-old girl with situs inversus totalis and DAA was presented. The baby was symptomatic with noisy breathing and coughing since birth. Double aortic arch was detected by echocardiography, barium esophagography, cardiac MRI and conventional angiography. The present report emphasis that vascular ring should have been evaluated in a patient with respiratory and/or feeding problems. Chest radiography, barium esophagography, echocardiography, multislice computed tomography, magnetic resonance imaging angiography and conventional cardiovascular angiography can be used for the diagnosis of DAA.