Little is known about the association between autosomal-dominant polycystic kidney disease (ADPKD) and coronary artery dissection (CAD). We suggest that the genetic disorder in ADPKD is the main cause of instable artery vasculature. Our case also shows that CAD can be missed in the acute phase. Therefore, we recommend additional investigation in patients with ADPKD presenting with acute chest pain. We report a case of a patient who developed a myocardial infarction due to a spontaneous dissection of the left anterior descending coronary artery. ADPKD was diagnosed during the additional investigation. The patient received medical management.