Congenital extrahepatic portal shunt is a rare congenital portal vein malformation, which is an anomalous portal-somatic shunt, manifesting as an anomalous anastomosis between the congenital portal vein and vena cava, with no portal vein blood flow to the liver for perfusion. Congenital extrahepatic anomalous portal shunt can be subdivided into two types according to the anomalous shunt between the portal vein and the vena cava, type I is the complete absence of portal blood flow to the liver, and type II is the partial perfusion of portal blood to the liver. There are few cases of this disease, so there is no unified understanding or mature treatment experience, and liver transplantation may be the only reliable method to treat this disease. The clinical manifestations of the disease are mainly related to the amount of shunt, the type of shunt, and the age of the child. patients with type I have many clinical symptoms, are detected early, and are often accompanied by congenital malformations of other organs such as multisplenic spleen and visceral ectasia, etc. Type II is usually a single malformation of the portal vein, with less severe clinical symptoms, is detected later, and is better tolerated by children with portal shunts, and the age of onset of the disease is mainly related to the amount of shunt. Guardians are advised to take children diagnosed with congenital extrahepatic portosystemic shunt to the doctor and cooperate with the doctor’s treatment in order to obtain a better prognosis.