Abstract: 　Objective　To explore the clinical manifestation, treatment and prognosis of anomalous left coronary artery from the pulmonary artery (ALCAPA). Method　The clinical data of patients with ALCAPA from January 2011 to September 2016 were retrospectively analyzed. All the patients were divided into infant group (<12 months) and child group (≥12 months). Results　In the 62 patients (38 infants and 24 children) with ALCAPA, the median ΔLVDD (actually measured LVDD – the normal upper limit of LVDD in this age group) of infant group and child group was 13.6 (8.4~17.5) mm and 8.5 (3.3~13.7) mm respectively. The mean LVEF of the infant group was 40.6±2.4 %, lower than that of the children group (59.0±2.9) %, and the difference was statistically significant (P<0.01). Sixty children were treated with surgery. The mean cardiopulmonary bypass duration of infant group (137.1±8.4 min) was longer than that of the children group (105.9±6.6 min), and the difference was statistically significant (P=0.010). The median mechanical ventilation time after operation in infant group (6 d, 3~7 d), was longer than that in the children group (2 d, 1~4 d), and the difference was statistically significant (P<0.01). The median follow-up time of 60 patients was 38 months. Six patients died within 1 month after surgery (5 cases in the infant group and 1 in the children group), and there were no deaths more than 1 month after surgery. In 22 cases, the flow rate of pulmonary valve increased rapidly within 1 week after operation (>1.8 m/s). Conclusion　ALCAPA is easily misdiagnosed as dilated cardiomyopathy. The clinical manifestations of infants were more severe than those of children. The mortality was high within 1 month after surgery, and the long-term prognosis was better.