Absent right superior vena cava in visceroatrial situs solitus is rare. It has been known the absent right superior vena cava is usually accompanied with persistent left superior vena cava. And this anomaly could be combined witk additional congenital heart disease and rhythm disturbances. We experienced one case of absent right superior vena cava accompanied by persistent left superior vena cava, secundum type atrial septal defect, and transient post-operative sinus node dysfunction.