Persistent left superior vena cava (LSVC) with absent right SVC (RSVC) is a rare congenital anomaly. If undetected, the condition may pose difficulties in central venous catheter insertion, pacemaker electrode insertion, and cannulation during cardiopulmonary bypass. We describe a case of persistent LSVC with absent RSVC, who was diagnosed to have bicuspid aortic valve with aortic stenosis.