Cardiac ischemia typically causes chest pain, variously radiating elsewhere. Convergence of cardiac nerve fibers on central pathways receiving somatic afferents from the head is likely to be responsible for the perception of cardiac ischemic pain as headache. A 47-year-old man was admitted to the emergency room of our hospital with a 2-hour history of occipital headache. Routine electrocardiography revealed monophasic ST-segment elevation in leads I and aVL and ST-segment depression in leads II, III, and aVF. During recording of lead VI, ventricular fibrillation occurred. Advanced life support was started immediately but failed to restore rhythm and cardiac function. Autopsy showed two-vessel disease with a ruptured plaque and total thrombotic occlusion of the proximal left anterior descending artery and 80% stenosis of the right coronary artery. In this patient, headache was the only symptom of myocardial ischemia. Anatomic convergence of cardiac nerve fibers on central pathways receiving somatic afferents from the head is likely to be responsible for the perception of cardiac ischemic pain as headache. Owing to the very rare occurrence of headache as a symptom of myocardial ischemia, diagnosis is difficult and requires a high degree of suspicion.