Abscence of right superior vena cava(SVC) in visceroatrial situs solitus is a rare(0.07% to 0.13%) congenital cardiovascular malformation, and little is known about the type and frequency of additional heart defects and arrhythmias. We reviewed previous publications and report the case of 8 month old male infant with absent right superior vena cava and persistent left superior vena cava and large secundum atrial septal defect, mild pulmonary valvular stenosis.