Polyarteritis nodosa (PAN) is a necrotizing form of vasculitis that affects small- and medium- sized vessels. Cases of ischemic heart disease involving coronary aneurysms in patients with PAN have been reported previously, but there have only been a few reports of coronary artery surgery for PAN-related coronary disease. A 46-year-old female with a history of PAN arrived at our emergency room due to cardiopulmonary arrest. After cardiopulmonary resuscitation, emergent coronary angiography was performed, because an electrocardiogram demonstrated ST segment elevation in leads V1 to V5. Coronary angiography revealed occlusion of the proximal right coronary artery and left anterior descending coronary artery as well as multiple coronary aneurysms. We performed emergent two-vessel coronary artery bypass grafting (the left anterior descending coronary artery and posterolateral branch of the circumflex system were treated with grafts from the left internal mammary artery and saphenous vein graft, respectively) under percutaneous cardiopulmonary support without cardiac arrest. The patient’s postoperative course was uneventful, and postoperative coronary angiography revealed that the bypass grafts were patent. A review of the literature and a discussion of this case are also presented.