Abstract: AIM To investigate the clinical features, diagnosis and treatment of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in infants. METHODS Initial and final diagnoses and surgery as well as follow-up results were reviewed in 29 ALCAPA patients who underwent surgical repair at Fuwai Hospital from June 2006 to January 2015. RESULTS Among the 29 infants with ALCAPA were 16 male patients and 13 female patients (mean age 0.75 years, range 2 months-1 year). Mean weight was 7.97 kg (range 4.8-11.5 kg) and mean left ventricle ejection fraction (EF) was 41.95% (range 18-66%). All patients had clinical symptoms of severe heart dysfunction. For confirmatory diagnoses, they underwent auxiliary examinations including electrocardiogram, echocardiogram (ECG) and ECG-gated 64-slice computed tomography. Mitral regurgitation was present in 19 patients: mild in ten patients, moderate in five patients and moderate to severe in four patients. Surgical methods were selected based on the locations of the anomalous left coronary artery origin from the pulmonary trunk and the distance between the left coronary artery and the aorta. Major surgical methods included the intrapulmonary tunnel (Takeuchi procedure) in one patient, direct reimplantation of the left coronary artery onto the aorta in 15 patients and reimplantation by pericardiac patch enlarge in 13 patients. Valvuloplasty was performed in nine patients with moderate and severe mitral regurgitation. Extracorporeal membrane oxygenation (ECMO) was used in three patients at the end of surgical repair. No hospital deaths and later deaths occurred. One to 80 months follow-up ［average (22±20) months］ in 24 patients (83%, 24/29) showed markedly alleviated symptoms in all 24 cases. CONCLUSION Early diagnosis and surgical treatment are important for ALCAPA patients. The establishment of a two-coronary system is the first choice of surgical therapy with satisfactory results of surgical repair of ALCAPA.