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Congenital absence of pericardium is an uncommon cardiac defect with variable clinical presentations. The detection of this malformation is clinically relevant because of potential complications such as fatal myocardial strangulation, myocardial ischemia and sudden death. Physical examination, chest radiograph and ECG are not helpful for the diagnosis. Echocardiography may accurately identify abnormalities in myocardial wall motion and in cardiac silhouette that may strongly suggest the diagnosis that is confirmed by magnetic resonance imaging (MRI) or computed tomography scan. A case presentation and a review of the literature with emphasis on the role of echocardiography are presented.