A 28-year-old woman with congenital aneurysm of the left atrial appendage was successfully treated by aneurysmectomy and is reported for its rarity. The exact diagnosis is relatively easy to make by contrast echocardiography, magnetic resonance imaging, and/or computerised tomographic angiography, provided the possibility is entertained. Because of supraventricular arrhythmias and systemic thromboembolism, surgical resection was deemed the best curative option to avoid further episodes and recurrence. Published reports detailing the diagnostic evaluation and surgical management for congenital left atrial appendage aneurysm are also discussed.