Congenital duodenal obstruction (CDO) is one of the most common anomalies presented in the neonatal period of intestinal obstruction, with an incidence of 1:10,000, and Preduodenal Portal Vein (PDPV) is a rare extrinsic cause of CDO. PDPV leads to CDO with a persistent vomiting, impairment of the oral feeding and weight loss, but other publications state an asymptomatic disease that may requires late surgical correction. Prenatal diagnosis of CDO, including PDPV, is evidenced by association of polyhydramnios with a dilated fluid-filled gastric and duodenum and a double-bobble image in the ultrasonography and permits a proper management of associated malformation, delivery, surgery and allows family counseling. PDPV is commonly associated to other intestinal obstruction such as extrinsic (annular pancreas, adhesive bands, malrotation, peritoneal bands, intestinal duplication), and intrinsic (atresia, web and stenosis), and also associated with cardiovascular anomalies, situs inversus, Down's syndrome and polysplenia. This is full term 5 days old newborn, 2865 g girl, without prenatal diagnosis of CDO. It was not still established PDPV until surgery procedure such as Duodenoduodenostomy (Diamond-Shape) and can guide Paediatric Surgeons that face high intestinal obstruction in the neonate with or without prenatal diagnosis. In the ambulatory follow up after 4 months, baby has developed very well, in breast-feeding associated with formula-feeding with a normal weight gain (average of 550 g/month).