We report a case of acute aortic dissection type A in a patient with situs inversus. A 33-year-old male, complaining of sudden chest pain, visited our institute. Contrast-enhanced computed tomography and echocardiography suggested Stanford type A acute aortic dissection and dextrocardia. Aortic root and ascending aortic replacement were successfully performed. Antegrade brain-isolated extracorporeal circulation was established. The aortic branch arteries were mirror-image reversed. Anatomic positional relationships and presence or absence of concurrent anomalies should be sufficiently investigated preoperatively in patients with dextrocardia. His postoperative course was uneventful and a postoperative computed tomographic scan confirmed a good result.