Abstract Purpose An anomalous aortic origin of the coronary artery (AAOCA) is a rare congenital heart disease. Some high-risk anatomical structures are at risk of inducing cardiogenic shock or even sudden death. This article summarizes our surgical experience with AAOCA in paediatric patients. Methods We retrospectively analysed the clinical data of 27 paediatric AAOCA patients admitted to the Department of Cardiothoracic Surgery in our hospital from July 2015 to June 2023 and summarized the surgical treatment experience and follow-up results. Results A total of 27 patients were included in this study, including 14 patients with an anomalous left coronary artery (ALCA) and 13 patients with an anomalous right coronary artery (ARCA). A comparison of clinical data between ALCA and ARCA patients revealed that the preoperative left ventricular ejection fraction (LVEF) in ALCA patients was significantly lower than that in ARCA patients (p