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We report four consecutive cases of Kommerell's aneurysm of an aberrant left subclavian artery in patients with a right-sided aortic arch and the results of a systematic review of the literature. In our cohort of patients, three had an aneurysm limited to the origin of the aberrant subclavian artery, causing dysphagia and cough, and one had an aneurysm involving also the distal arch and the entire descending thoracic aorta, causing compression of the right main-stem bronchus. A left subclavian-to-carotid transposition was performed in association with the intrathoracic procedure, and a right thoracotomy was used in all patients. One of the patients underwent surgery with deep hypothermia and circulatory arrest, and the others with the adjunct of a left-heart bypass. The repair was accomplished with an interposition graft in two patients and with endoaneurysmorrhaphy in the others. The postoperative course was complicated by respiratory failure and prolonged ventilation in one patient, and one patient died because of severe pulmonary emboli. The survivors are alive and well at a follow-up of 1 to 3 years. Only 32 cases of right-sided aortic arch with an aneurysm of the aberrant subclavian artery have been reported: 12 were associated with aortic dissection, and 2 presented with rupture. Surgical repair was accomplished in 29 patients. A number of operative strategies were described: right thoracotomy, bilateral thoracotomy, left thoracotomy with sternotomy, sternotomy with right thoracotomy, and left thoracotomy. In only 12 cases was the subclavian artery reconstructed. We believe that a right thoracotomy provides good exposure and avoids the morbidity associated with bilateral thoracotomy or sternotomy and thoracotomy. We feel that a left subclavian-to-carotid transposition completed before the thoracic approach revascularizes the subclavian distribution without increasing the complexity of the intrathoracic procedure.