A 1-day-old female infant presented with vomiting immediately after feeding and shortness of breath after birth. Esophagography revealed external compression of the esophagus. Echocardiography showed a right aortic arch with mirror image branching of brachiocephalic vessels without intracardiac anomalies. Left ventriculography confirmed the echocardiography findings and revealed a tenting of the proximal part of the left subclavian artery and a blind pouch of Kommerell diverticulum at the descending aorta. The patient underwent surgery, which revealed a complete vascular ring compressing the trachea and the esophagus. The ring was formed by a right aortic arch, atretic left arch and left ligamentum arteriosum. Following surgical division of the ligamentum arteriosum and the atretic left arch, the symptoms subsequently improved.