We report a case of end stage renal disease patient who displayed a persistent left superior vena cava (PLSVC) after placement of hemodialysis (HD) catheter through left internal jugular vein, as revealed by routine post-procedure X-ray chest. The diagnosis of PLSVC was confirmed by arterial blood gas, two-dimensional echocardiography, computed tomography thorax and angiographic examination. This anomaly is rather rare; few studies on safety of PLSVC for HD have been reported. The catheter was uneventfully used for HD for 2 months with careful continuous monitoring and removed after arteriovenous fistula was successfully cannulated. Physicians who place HD catheters in the left jugular/subclavian vein should be aware of the existence of PLSVC.