Congenital Complete Absence of Pericardium Presenting with Chest

Congenital absence of the pericardium is a very rare anomaly which has an incidence of 1 in 14000 cases in autopsy studies. Defects in the pericardium are either partial or complete with complete defects having a much benign course than partial defects. Partial defects are associated with complications like strangulation of a herniated cardiac chamber or coronary artery stenosis from external compression. Absence of the left pericardium is the most frequently encountered type of defect which accounts for 2/3 of the cases. We herein report a case of complete absence of the entire pericardium which is an extremely rare subtype with associated Cardiac magnetic resonance images.