Persistent left and absent right superior vena cava is a rare congenital anomaly, which is usually asymptomatic. Persistent left superior vena cava (PLSVC) is generally coexistent with right superior vena cava (RSVC), but rarely associated with absent RSVC. Herein, we report two children referred to our department because of ventricular septal defect. We determined PLSVC with absent RSVC during the angiography.