Background: Congenital absence of pericardium is a rare cardiac disorder with a reported incidence of less than 1 in 10,000. Although most of the cases are of little clinical significance, some of them are associated with serious complications, including risk of herniation and strangulation or coronary artery compression. Detailed Case Description: We report a case of a 36-year-old male referred for routine cardiovascular examination. He had a medical history of a heart murmur since childhood. Electrocardiogram (ECG) revealed sinus rhythm, normal axis, poor R-wave progression in the precordial leads and repolarization abnormalities with negative T waves in leads V1–V4. On 2D transthoracic echocardiography (TTE), an unusual heart position was noted with poor image quality from the standard acoustic windows. The parasternal long axis view gave the impression of right ventricular dilatation. The findings raised the suspicion of left to right shunt and possible atrial septal defect. For further evaluation, the patient was referred for cardiac magnetic resonance which demonstrated complete left-sided absence of the pericardium. Discussion: Due to indistinct and atypical symptoms and lack of clinical awareness, pericardial congenital absence is frequently misdiagnosed. Patients may complain of atypical chest pain. Patient’s history and physical examination are often nonspecific. In cases with complete pericardial absence, ECG findings may include right axis deviation, right bundle block and sinus bradycardia. Echocardiography findings are also not characteristic, but some may raise the clinical suspicion of this diagnosis. The imaging modalities of choice are computed tomography and cardiac magnetic resonance. Treatment depends on the type of defect and clinical symptoms.