Congenital absence of the pericardium should not be considered rare. Of the 87 cases reviewed, congenital absence of the left pericardium (partial and total types) accounted for the vast majority of cases. Congenital absence of the right pericardium (partial and total types) is relatively unusual and absence of the entire pericardium (left and right sides) as well as defects at the base of the pericardium are rare. The appearance of complete absence of the left pericardium as seen with conventional plain film roentgenography is characteristic. The presence of right axis deviation on the electrocardiogram, a systolic precordial murmur, as well as displacement of the point of maximal cardiac impulse may be present in various combinations. The appearance of partial left on partial right pericardial defects as seen with conventional plain film roentgenography is not characteristic and requires a high index of suspicion for diagnosis. Angiocardiography is the method of choice for diagnosis. Cinefluoroscopic demonstration of the free rolled edge of the remaining pericardium is presented.