The presence of an aberrant right subclavian artery (ARSA) arising from the proximal descending aorta is a relatively common anomaly of the aortic arch. These vessels may be involved in aortic dissections, either as the site of the primary intimal tear or as a dissected aortic branch, subject to flow limitation and future aneurysmal degeneration. We report a case involving a ruptured acute type A aortic dissection originating from ARSA. The coincidence of an ARSA with a complicated acute type A aortic dissection is extremely rare, as in the present case. This case was diagnosed preoperatively by computed tomography angiography (CTA), which is of great value for precise planning of surgical strategy in such cases. The aim of the case report is to make the clinicians aware of ARSA probably being accompanied with aortic dissection, so as to diagnose it and manage it rapidly and appropriately.