The case of a 41-year-old man who developed an aneurysm in his aberrant left subclavian artery is described. The patient had a right aortic arch. After a successful aortosubclavian artery bypass, symptoms due to brain ischemia disappeared. This is a very rare disease that is sometimes associated with an aortic anomaly, therefore the optimal therapeutic procedure need to be carefully selected, including the operative indications and approach.