Polysplenia syndrome, also known as left isomerism, is a type of heterotaxy syndrome where there are multiple spleens (congenitally) as part of left–sided isomerism. Polysplenia is characterised by multiple splenules without a parent spleen. The most common associated feature is inferior vena cava interruption with azygous or hemiazygous continuation. Rare associations of polysplenia syndrome includes preduodenal portal vein (PDPV) and short truncated pancreas. PDPV is a rare developmental anomaly in which the portal vein runs at the ventral side of the duodenum instead of at an intrapancreatic location. Understanding of this anomaly is of considerable surgical importance, because it may cause unintended portal vein injury during operations involving the gall bladder or duodenum. We recently and incidentally detected a PDPV and short truncated pancreas associated with polysplenia syndrome in a 22–year–old female patient who complained of dry cough. We report here the Multidetector–row CT findings of a rare case of polysplenia with PDPV, short truncated pancreas and other anomalies.