BackgroundCongenital left atrial appendage aneurysm (LAAA) is a rare cardiac anomaly with potentially serious complications, including life-threatening systemic thromboembolism, atrial tachyarrhythmia, and cardiac dysfunction. Currently, early surgical intervention is generally recommended to prevent these complications.Case presentationWe present a case of congenital giant LAAA in a female patient who successfully completed pregnancy and underwent caesarean section with no obvious complications. Surgical resection of the LAAA was performed 3 years later, at the onset of chest pain resulting from compression of adjacent cardiac structures by the LAAA.ConclusionSurgical resection is recommended for the majority of patients with LAAA because of potential LAAA-related severe outcomes. However, clinical monitoring may be an optional strategy for asymptomatic patients without intra-atrial thrombus or other complications. Precise evaluation with echocardiography and brain magnetic resonance imaging is valuable for the subsequent management of LAAA.