We report a clinical case of a persistent left superior vena cava discovered in a 50-year-old female patient when a port-a-cath was inserted. This already seldom malformation was associated with an arteria lusoria and polysplenia with left inferior vena cava with hemiazygos continuation, right-sided stomach, short pancreas, preduodenal portal vein and intestinal malrotation, but without any cardiac abnormalities.