Congenital absence of the pericardium is a rare abnormality that can be diagnosed by cardiac imaging procedures. A 49-year-old male needed medical attention due to the appearance of palpitation with a systolic murmur, and a notable aortic arch deviation was seen in the chest X-ray. In the echocardiogram, a poor echo window was detected. A cardiac magnetic resonance imaging (MRI) showed a rare concomitant anomaly of partial absence of the pericardium including a rare defect of the right-sided aortic arch. Using cardiac MRI, the pericardium can be easily visualized, and thus, its absence more easily detected, aiding appropriate clinical decision-making.