A coronary arteriovenous fistula (CAVF) is an unusual anomaly in adults, and a CAVF arising from the left main and anterior descending arteries, presenting with acute myocardial infarction and cardiogenic shock, has not been reported. A 62 year-old male suffered from chest discomfort of 3-hour duration. His blood pressure at the emergency room was 70/40 mmHg, and an electrocardiogram showed marked ST segment depression in leads II, III and aVF, and from V3 to V6. The levels of creatinine kinase-MB and troponin I were 65 U/L and 4.36 ng/mL, respectively. A diagnostic coronary angiogram revealed no significant coronary stenosis, but large coronary arteriovenous fistulae originating from the left main and anterior descending coronary arteries to the main pulmonary artery. Surgical ligation for a CAVF was successfully performed, and a one-month follow-up coronary angiogram showed completely ligated fistulae.