Congenital left atrial appendage aneurysm is an extremely rare anomaly. It is seen at any time ranging from fetal to ninth decade of life. Patients with left atrial appendage aneurysm may be asymptomatic, or present with dyspnea, arrhythmias, thromboembolic phenomenon. We report a case of prenatal diagnosis at 26 weeks of gestation , postnatal management and outcome of left atrial appendage aneurysm.