A fully documented case of complete absence of the left pericardium in a 44-year-old male is presented. Complaints of acute chest pain were caused by strangulation of the heart between the pulmonary ligament and the diaphragm. Preoperative electrocardiogram, chest X-ray and echocardiography showed 'classic' signs as described in the literature. A CAT scan suggested absence of the left pericardium, the definitive diagnosis was made with the aid of thoracoscopy. This case of total absence of the left pericardium is the first documented one requiring thoracic surgery. The defect was closed through a left-lateral thoracotomy using an allograft of pig pericardium.