Objective This study focuses on determining concomitant congenital heart disease in patents with persistent left superior vena cava. Methods The data of ulrasonic diagnosis and clinical date of 1676 children with persistent left superior vena cava were analyzed between 2013 and 2017. Result The incidence of persistent left superior vena cava was 0.79% (1676/212309) in all patients, and type I permanent left superior vena cava is the most common accounting for 9.7% (1671/1676). The incidence of persistent left superior vena cava was 3.0% (866/28403) in children with congenital heart disease. A total of 1676 patents with persistent left superior vena cava, 866 (51.7%) of whom were diagnosed with congenital heart disease. Of all the 1676 cases, the common concomitant congenital heart disease were ventricular septal defect (202, 23.3%), atrial septal defect (149, 17.2%), tetralogy of Fallot (73, 8.4%), patent ductus arteriosus (58,6.7%), isolated aortic coarctation and the aortic coarctation complex (52, 6.0%), endocardial pad defect (34,4.0%), double outlet right ventricle (29, 3.3%). Conclusion Increased awareness about the associaton of certain congenital heart disease with persistent left superior vena cava and a careful echocardiographic examinaton can facilitate the diagnosis of left superior vena cava. In additon, precise prior diagnosis of persistent left superior vena cava and its associated deformities can provide a reliable basis of the treatment.