Preduodenal portal vein (PDPV) is a rare congenital vascular anomaly in which the portal vein passesanterior to the duodenum rather than posteriorly. Generally asymptomatic, PDPV may rarely cause aduodenal obstruction in the newborn. It is usually associated with gastrointestinal tract, cardiac,pancreatic, as well as biliary tract anomalies or may, occur as a single isolated malformation. Tillnow, only a few cases have been reported with duodenal obstruction and associated anomalies. Thepresent study report one such case of PDPV with multiple congenital anomalies. A full-term, one-day-old baby who had an antenatal history of polyhydramnios, presented to us with abdominaldistension and non-bilious vomitings soon after birth. Surgical exploration revealed a hugely dilatedstomach, multiple Ladd bands, malrotation of the small intestine, preduodenal portal vein, and anannular pancreas causing external duodenal compression. Intraoperative recognition of PDPV isimportant because iatrogenic injury during surgery can cause profuse hemorrhage from the vein andmay cause damage to the biliary tract or duodenal wall. Prompt evaluation of associated cardiacanomalies is important before surgery. Duodenostomy anterior to the portal vein is the definitivetreatment. Studies in animal models (AKR/J mice) have shown an autosomal recessive mode ofinheritance.