OBJECTIVE: To study the diagnosis and surgical treatment of congenital coronary artery fistula. METHODS: Twenty two children with this disorder confirmed by X ray, selective angiography and echocardiography, were treated surgically.RESULTS: The coincidence rate of diagnosis by selective angiography (n=19) and echocardiography (n=17) was 94.2% , while that by selective angiography and operation was 99.8% . The operation was successful in 21 patients, and only one died from the low cardiac output syndrome. Of the 21 cases, 19 kept cardiac functional class I during a 15 month to 23 year follow up. CONCLUSIONS: With technical skills and experience of echocardiography, diagnosis of congenital artery fistula is easy. Nevertheless definite diagnosis has to be confirmed by selective angiography or MRI. Surgical treatment is very effective and safe for those who have a definitive diagnosis. In the operation, one should ensure closure of the coronary artery fistula outlet to avoid residual shunting and ensure adequate blood supply to the distal coronary artery.