OBJECTIVE: The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery abnormality. This study aimed to investigate the clinical characteristics, diagnosis and treatment of the disorder. METHODS: The medical data, including common clinical presentations, the findings of the digital subtraction angiogram (DSA), echocardiograms and the electrocardiograph (ECG), surgical records and the outcome, of 10 children with ALCAPA between June 2001 and February 2005 were retrospectively reviewed. RESULTS: Diaphoresis, fantod, breathlessness and difficult feeding were commonly presented in the patients with ALCAPA. ECG, echocardiography and DSA examinations showed characteristic changes in these patients. The Takeuchi procedure was performed in 4 patients, direct aortic reimplantation in 4 cases and aortic reconstruction in 2 cases. After operation the symptoms of 8 patients were improved but 2 died. CONCLUSIONS: ALCAPA patients have characteristic clinical manifestations and lab findings. This is helpful in making an early diagnosis of ALCAPA. Early surgical treatment can improve the patients' outcome.［Chin J Contemp Pediatr, 2007, 9 (1):25-27］