Variations in the course of the blood vessels are often incidental findings during clinical examination. A persistent left superior vena cava (LSVC) is really not rare (healthy individuals, 0.3-0.5%; patients with congenital heart disease, 4%) and serious complications have been described during catheterization in adults with LSVC (shock, cardiac arrest, angina). Therefore variations of the superior vena cava should be considered, especially when central venous catheterization via the subclavian or internal jugular vein is difficult. We describe the embryogenesis and the anatomic variations of persistent LSVC. Subsequently we suggest a classification of superior vena cava according to the positioning of a central venous catheter on the chest radiograph: type I, normal anatomy; type II, only persistent left superior vena cava; type IIIa, right and left superior vena cava with connection; type IIIb, right and left superior vena cava without connection. This classification is illustrated by four clinical cases.