Acute myocardial infarction (AMI) is one of the major causes of mortality and morbidity worldwide. In Central Europe, causes of AMI other than atherosclerosis are unusual. Coronary artery vasculitis is one potential non-atherosclerotic process causing AMI. Herein, the authors depict a very rare case of AMI as a clinical manifestation of polyarteritis nodosa (PAN), a necrotizing systemic vasculitis. A 49-year-old male patient presented to our clinic with abdominal pain and markedly elevated concentrations of C‑reactive protein, creatinine and high-sensitivity cardiac troponin T. Electrocardiography showed new repolarization abnormalities in aVF and III. Besides PAN-typical angiographic findings, including bilateral renal artery microaneuryms as well as different arterial occlusions, coronary angiography displayed a complete thrombotic occlusion of the right coronary artery without any other coronary pathology. The present case report demonstrates AMI as very rare but deleterious complication in patients suffering from PAN, and highlights that this life-threatening event can occur even at a very early stage of PAN-related coronary affection.