Background <p>Double aortic arch (DAA) is a rare congenital aortic arch anomaly that is usually identified in infancy because of symptoms related to a vascular ring. Acute type A intramural hematoma (IMH) with a focal intimal tear in an adult with DAA is extremely rare, and no consensus has been established regarding emergency management.</p> Case presentation <p>Seventy-three year-old woman presented with burning pain in the throat and suprasternal notch and was initially suspected of having acute coronary syndrome. Computed tomography angiography (CTA) of the whole aorta and supra-aortic vessels demonstrated a double aortic arch, with the right common carotid artery and right subclavian artery originating from the right arch and the left common carotid artery and left subclavian artery originating from the left arch. After preoperative evaluation, emergency surgery was performed through a median sternotomy. Intraoperatively, a focal intimal tear approximately 2&#xa0;cm in length was identified in the ascending aorta. Given the complex branching anatomy of the double aortic arch, right femoral artery cannulation was used to establish cardiopulmonary bypass in order to minimize invasive manipulation of the arch. Resection of the diseased ascending aorta and graft replacement were performed, and the potential false lumen at the aortic root was obliterated using the adventitial inversion technique. The prosthetic graft was wrapped with bovine pericardium, and the double aortic arch was not addressed during the same operation. The patient experienced recurrent perioperative hypoxemia and was extubated 17&#xa0;h after surgery following respiratory support, lung-protective management, and anti-inflammatory treatment. She was discharged on postoperative day 12. Approximately 1&#xa0;month later, she was readmitted with chest pain, and CTA revealed a newly developed dissection in the proximal right aortic arch. The family declined reoperation, and the patient was subsequently lost to follow-up.</p> Conclusions <p>In patients with DAA complicated by acute type A IMH with a focal intimal tear, limited ascending aortic replacement in the emergency setting may reduce surgical trauma; however, it may leave a high-risk residual arch segment and increase the risk of clamp-related injury or insufficient resection margins. Perioperative airway compression caused by the vascular ring should be assessed using imaging, and one-stage or staged reconstruction should be planned according to the patient's condition. Strict postoperative blood pressure control and close follow-up are essential to reduce the risk of recurrence.</p>