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Abstract Background An aberrant right subclavian artery which arises from the proximal descending aorta may result in aortic dissection. The dissection may occur at either the site of the primary intimal tear or from an aortic branch. These conditions may lead to blood flow limitation and possible aneurysmal degeneration in the future. Case presentation We described the clinical presentation and management of a 54-year old patient diagnosed with a rare case of an aberrant right subclavian artery with Stanford Type B aortic dissection. A hybrid surgical approach was successfully performed and the patient had an uneventful recovery. Conclusion Even though aortic dissection is often an incidental finding, this case highlighted that in rare situations, it can be associated with an aberrant right subclavian artery. It is important to disseminate this association as it has profound diagnostic and therapeutic implications in safeguarding the clinical outcomes of patients with such condition.