Cystic adventitial disease is rare, but it is one of the well-recognized causes of non-atherosclerotic arterial stenosis or obstruction. Despite one of its most common symptoms being chronic intermittent claudication, it may be misdiagnosed as arterial embolism when presented with acute ischemic symptoms. Surgical resection is recommended because of recurrence or a low success rate with aspiration or endovascular stent. We performed resection and repair with autologous vein patch for cystic adventitial disease of the popliteal artery of a 57-year-old man presenting with pain, pallor, and paresthesia, without any postoperative complications or recurrence.