<p>The etiology of right-sided aortic arch anomalies has not been well elucidated yet. This highly rare anomaly is typically asymptomatic and can often be misdiagnosed. Symptoms may arise due to the anatomy of the right-sided aortic arch causing compression of mediastinal structures, or as a result of aneurysmal disease or dissection. Symptoms and signs of the condition originate from the compression of adjacent tissues and organs by the retroesophageal course of the aberrant right subclavian artery and, if present, aneurysm of the descending aorta. In this article, we present a 45-year-old male patient with chronic Type A aortic dissection with a Type-II right-sided aortic arch treated with hybrid approach. In conclusion, although rare, it may present with a clinical picture characterized by prominent compression symptoms such as tachypnea, dyspnea, and dysphagia and, therefore, right-sided aortic arch should be considered in the differential diagnosis. Timely and accurate diagnosis, appropriate surgical, hybrid, or endovascular interventions, and close postoperative multidisciplinary follow-up are of utmost importance to ensure survival.</p>