Polycythemia vera is a chronic progressive myeloproliferative disease characterized by increased circulating red blood cells, and the hyperviscosity of the blood can lead to an increased risk of arterial thrombosis. In a previous survey regarding postoperative outcomes in polycythemia vera patients, an increased risk of both vascular occlusive and hemorrhagic complications have been reported. Aortic surgery involving cardiopulmonary bypass may be associated with the development of a coagulopathy, and as a result, the occurrence of thrombotic complications should be avoided after coronary anastomosis. Thus, optimizing the hemostatic balance is an important concern for anesthesiologists. However, only a few cases of anesthetic management in polycythemia vera patients undergoing concomitant aorta and coronary arterial bypass surgery have ever been reported. Here, we experience a polycythemia vera patient who underwent an emergency repair of a type-A aortic dissection and concomitant coronary artery bypass grafting, and report this case with a review of the relevant literature.