Persistent left superior vena cava (PLSVC) is a rare anatomic variant that has a significant effect on the structure of the heart and venous system with clinical implications that are far-reaching. The presence of this variant is relevant to central venous catheter insertion, cardioverter-defibrillator placement, coronary artery bypass grafting, and numerous other medical procedures. In this report, we describe a rare case of PLSVC with a connection to the azygos system; notably, the vast majority of PLSVCs connect to the coronary sinus. We also discuss the anatomic and anesthetic considerations for individuals with this uncommon variant.