Autosomal dominant polycystic kidney disease (ADPKD), characterized by renal cyst formation, is known to cause such vascular abnormalities as arterial dilatation and dissection. However, spontaneous coronary artery dissection (SCAD) is observed only rarely in patients with ADPKD. We report a patient with ADPKD who developed SCAD and presented with acute myocardial infarction. Her coronary angiography showed a long spiral dissection of the left anterior descending coronary artery. She underwent successful coronary angioplasty with insertion of 3 drug-eluting stents. To the best of our knowledge, this is the first reported case of percutaneous coronary intervention for coronary dissection in a patient with ADPKD. The pathophysiological characteristics of vascular complications in patients with ADPKD are discussed. Polycystins are strongly expressed in human adult vascular smooth muscle cells, and the vascular abnormalities in patients with ADPKD may be related to altered expression of polycystins. Because early recoginition and prompt efforts at mechanical reperfusion, if indicated, are crucial for successful management of SCAD, it would be worthwhile to consider SCAD in the differential diagnoses of acute coronary syndrome in patients with ADPKD.