Persistent left superior vena cava with absent right superior vena cava is a rare congenital anomaly with few cases reported to date. To the best of our knowledge, the current case is the first one to be reported from New Zealand. The condition may present difficulties in central venous catheterization, pacemaker electrode insertion and cannulation during cardiopulmonary bypass. We describe a case of persistent left superior vena cava with absent right superior vena cava, who presented with aortic stenosis (bicuspid aortic valve) and rhythm disturbances.