BACKGROUND: A Kommerell diverticulum (KD) is an aneurysmal remnant of the dorsal fourth aortic arch. This can be an independent cause of tracheoesophageal compression. We previously reported resection of the KD with left subclavian artery transfer to the left carotid artery for recurrent symptoms in patients with a right aortic arch, left ligamentum, and retroesophageal left subclavian artery after prior ligamentum division. In 2001 we began resecting the KD and transferring the left subclavian artery to the left carotid artery in selected patients as a primary operation. METHODS: From 2001 to 2011, 20 patients have had primary excision of a Kommerell diverticulum. Diagnosis was with computed tomographic scan (n = 14) or magnetic resonance imaging (n = 6) and bronchoscopy. Sixteen patients had a right aortic arch and 4 had a double aortic arch (right arch dominant). All patients were approached through a left thoracotomy. Fifteen patients had simultaneous division and reimplantation of the left subclavian artery into the left carotid artery. RESULTS: Mean age at operation was 9.1 +/- 6.5 years (range 1.5 to 29.1 years). Symptoms included cough, wheezing, stridor, dysphagia, and dyspnea on exertion. Selection criteria included KD greater than 1.5 times the size of the left subclavian artery and posterior pulsatile compression of the trachea on bronchoscopy. There were no complications related to subclavian artery transfer. No patient required a blood transfusion. No patient had a recurrent laryngeal nerve injury or chylothorax. The mean hospital stay was 4.3 +/- 2.5 days. All patients had resolution of their preoperative airway and esophageal symptoms. CONCLUSIONS: In selected patients with a vascular ring we now recommend resection of the associated Kommerell diverticulum and transfer of the retroesophageal left subclavian artery to the left carotid artery as a primary procedure. This strategy requires comprehensive and precise preoperative imaging with either computed tomography or magnetic resonance imaging.