Autosomal dominant polycystic kidney disease (ADPKD) may present with a variety of extrarenal manifestations. Only 1 case of spontaneous coronary artery dissection has been reported so far in ADPKD. Here we report a case of coronary artery dissection occurring in a 38-year-old woman with ADPKD and an unremarkable medical history. She was admitted to the intensive care unit with acute chest pain. Anterior myocardial infarction was diagnosed by ECG; the patient was treated with thrombolysis with tenecteplase and aspirin, heparin and carvedilol. Then she was referred to the cardiac catheterization laboratory. Coronary angiography revealed a long linear dissection in the middle left descending coronary artery (DCA) with TIMI 1 distal flow. Complete vessel recanalization was achieved by positioning 2 drug-eluting and overlapped stents in the middle DCA and a further proximal stent due to residual proximal dissection, resulting in a TIMI 3 distal flow. There were no procedural complications, and the patient was discharged on day 8. In conclusion, spontaneous coronary artery dissection is a rare cause of myocardial ischemia and infarction. Myocardial infarction in patients without any risk factors for coronary atherosclerosis or in young patients should prompt a diligent search for a possible spontaneous coronary artery dissection. It is suggested that ADPKD may be a predisposing factor for spontaneous coronary artery dissection.