Objective To comprehend and analyze the characteristics of congenital quadricuspid aortic valve in echocardiogram and the matters needing attention in its diagnosis. Methods The clinical data of a case of congenital quadricuspid aortic valve were analyzed retrospectively, combined with the results of 113 cases published in Chinese and foreign journals from 2000. Results From the echocardiography fin dings of 113 patients, if the aortic valve is closed and the regurgitation, we should pay more attention to observe the number, distribution and development of aortic valve, so we can be timely and clearly diagnose some diseases, and reduce misdiagnosis. Conclusion When the aorta is closed or others , the echocardiogram should be carefully probed, especially the short axis view of the aortic valve, in order to prevent misdiagnosis.