Persistent left superior vena cava can present as a benign anatomic finding or as a pathologic entity, causing significant hemodynamic manifestations. Its presence can also promote modifications in the surgical techniques used for treatment of other cardiac pathologies. When persistent left superior vena cava constitutes a pathologic entity that needs to be surgically addressed, this can be approached either by intracardiac or extracardiac procedures. Extracardiac procedures appear simpler and reproducible. A previously reported technique for extracardiac approach of left superior vena cava using an enlarged left superior vena cava-right atrial appendage anatomosis is reviewed.