Persistent left superior vena cava is an extremely rare venous anomaly affecting 0.5% to 2% of the general population. Persistent left superior vena cava with absent right superior vena cava, also termed as “isolated persistent left superior vena cava.” Persistent left superior vena cava, without associated cardiac anomalies, is usually innocuous. Its discovery, however, has important clinical implications. It can pose clinical difficulties with central venous access, hemodialysis catheter placement, and pacemaker implantation. We hereby present a case of persistent left superior vena cava that was incidentally encountered after the placement of a hemodialysis catheter through the left internal jugular vein. This case highlights the pertinent radiologic findings and emphasizes the importance of familiarity to such an anatomic anomaly.