A bicuspid aortic valve is one of common congenital cardiac anomaly. But the septic embolism from bicuspid aortic valve into the left anterior descending artery (LAD) resulting in acute inferior myocardial infarction is very rare. A twenty eight year-old man suffered from severe chest pain and fever of four-week's duration. Acute inferior myocardial infarction was diagnosed on electrocardiogram. Transesophageal echocardiogram demonstrated vegetation on the bicuspid aortic valve directed into left coronary artery ostium, and diagnostic coronary angiogram revealed round filling defect within the distal LAD. He underwent operation for aortic valve replacement, which indicated vegetated bicuspid aortic valve directed into the ostium of left coronary artery.