Left atrial appendage aneurysm (LAAA) is a rare pathologic entity that can be congenital or, more frequently, acquired. Its complications include arrhythmias and thromboembolic events, palpitations, hiccups, chest pain, dyspnea, and myocardial infarction. LAAA can be isolated or associated with other congenital anomalies such as atrial septal defect, ventricular septal defect, anomalous renal artery, Noonan syndrome, and Hurler‐Scheie syndrome. We report a rare case of giant LAA with intra‐aneurysmal thrombus diagnosed by contrast‐enhanced echocardiography.