Congenital anomalies of the pericardium are extremely rare and often difficult to diagnose. We describe a 66-year-old woman presenting with multivessel coronary artery disease and right heart insufficiency complicated by an extremely left-rotated heart due to a congenital absence of left pericardium. A multidetector row computerized tomographic study was performed to evaluate the torsion of the heart as well as the physical relationship between the left internal thoracic artery and the left coronary system. Off-pump coronary surgery was scheduled. Both the operative and postoperative courses were uneventful and the patient was discharged after 11 days.