Left atrial appendage aneurysm (LAAA) is a rare congenital anomaly which can be associated with cardiovascular morbidity and mortality by predisposing to atrial tachyarrhythmias, thromboembolism and cardiac failure. In this case report a patient presenting with supraventricular arrythmias and chest pain due to LAAA is presented. Surgical treatment was performed through median sternotomy with cardiopulmonary bypass through which the giant aneurysm of the left atrial appendage, extending to the apex of the left ventricle was surgically managed successfully. Surgical treatment is curative and proposed as the main treatment modality regardless of the presence or absence of symptoms.