Background: Persistent left superior vena cava (PLSVC) is rare but important congenital vascular anomaly. It results when the left superior cardinal vein caudal to the innominate vein fails to regress. It is most commonly observed in isolation but can be associated with other cardiovascular abnormalities including atrial septal defect, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, and cortriatriatum. Objective: Aimof this case presentation was to describe a rare case of persistent left superior vena cava that is associated with atrial septal defect and partial anomalous pulmonary venous drainage. Also, how to suspect and then confirm the presence of PLSVC by echocardiographic examination, and to know the role of multi-slice CT and cardiac MRI for detection of other cardiac and extra-cardiac anomalies. Patients and methods: Male patient aged 32 year complaining of shortness of breath for about 1 year, echocardiographic examination revealed dilated coronary sinus and injection of agitated saline indicated the presence of persistent left superior vena cava. PLSVC may be associated with other anomalies so further evaluation by multi-slice CT was done to confirm the diagnosis of PLSVC as well as sinus venosus ASD and partial anomalous pulmonary venous drainage so surgical consultation was done for surgical correction. Conclusion: Diagnosis of PLSVC is important, as it is often associated with other anomalies as atrial septal defect and partial anomalous pulmonary venous drainage, which lead to pulmonary hypertension and need surgical correction before the occurrence of pulmonary hypertension.