Aberrant right subclavian artery (ARSA) is a congenital anomaly that usually does not produce symptoms. However, patients with symptoms may require surgical intervention. Surgical procedures consist of division of ARSA from the descending aorta and reimplantation to the right common carotid artery or ascending aorta. Specific anesthetic management includes invasive monitoring of blood pressure in both radial arteries, monitoring of adequate cerebral perfusion, and ventilatory strategy to facilitate surgical exposure. Although many reports are available regarding its surgical treatment, there are few reports, to the author's best knowledge, that describe anesthetic management for its surgical correction. This case report will focus on important aspects of the anesthetic management of patients with ARSA.