Objective To study the value of echocardiography for diagnosis and to discuss the features of the anterior descending artery originating from the right coronary artery in tetralogy of Fallot (TOF). Methods The echocardiographic characteristics of the anterior descending artery originating from the right coronary artery in TOF in five cases were compared with the results of cardiac catheterization and operation. The diagnostic features of echocardiography were summarized. Results Besides the five cases had the echocardiographic characteristics of TOF, other echoeardiographic characteristics were: anterior descending artery originating from the right coronary artery and crossing the right ventricular outflow tract were visualized in suprasternal short-axis view. Right coronary artery became larger than normal. There was no branch could be see at beginning of the main left coronary artery. The echocardiographic characteristics of the anterior descending artery originating from the right coronary artery in TOF cases were correspondent to the results of cardiac catheterization and operation. Conclusions There is high accuracy of echocardiography to diagnosis the anterior descending artery originating from the right coronary artery in TOF. Coronary artery abnormalities should be alerted in TOF.