Hypoplastic coronary artery disease (HCAD) is a rare condition that may lead to myocardial infarction (MI) and sudden death. We discovered HCAD in a young man who developed chest pain after heavy drinking and who was found to have suffered an MI. His ECG showed ST-segment elevation with Q waves in the anterior leads, and echocardiography revealed apical dyskinesia with moderate left ventricular (LV) dysfunction. Coronary angiography showed hypoplasia of the left anterior descending (LAD) artery. 99mTc-tetrofosmin-gated myocardial perfusion scintigraphy showed a large, fixed perfusion defect in the anteroseptal and apical segments. Sixty-fourslice cardiac CT and cardiac MR imaging demonstrated thinning of the apical wall with calcification and delayed enhancement, supporting the diagnosis of long-standing MI. The patient was discharged symptom-free on medication for ischemic heart failure two weeks after admission. Although HCAD is very uncommon, it should be considered in children and young adults who suffer MI or sudden cardiac death.