【】 Objective: To explore the prenatal diagnostic features of fetal dilated coronary sinus of which combined with cardiac and extracardiac malformation. Methods: This was a retrospective review of prenatally diagnosed cases with dilated coronary sinus in Guangzhou Women and Children’s Medical Center and underwent fetal ultrasound between July 2011 and April 2015. Results: 202 cases diagnosed fetal dilated coronary sinus were studied. There were 194 cases assembly of persistent left superior vena cava, of which 52 fetuses with other cardiac malformations, 17 cases of extracardiac defects and 7 cases of both cardiac and extracardiac defects; There were 4 cases of anomalous pulmonary venous drainage of which one fetus with other cardiac malformations; There was only one case of unroofed coronary sinus and meanwhile with cardiac anomalies; One case is not only of persistent left superior vena cava but also anomalous pulmonary venous drainage; The last two cases with no other anomalies. Ventricular septal defect and pulmonary stenosis and tetralogy of Fallot are the most common cardiac abnormalities and single umbilical artery is the most common extracardiac malformation. Conclusion: Persistent left superior vena cava is the most reason of dilated coronary sinus and the most common cardiac abnormalities are: ventricular septal defect and pulmonary stenosis and tetralogy of Fallot. Prenatal ultrasound has an important diagnostic value in fetal dilated coronary sinus and of which combined with cardiac and extracardiac malformation.