Abstract: Objective To discuss the diagnosis, clinical features, and treatment for prehepatic portal hypertension and investigate the efficacy of surgery for this disease.Methods A retrospective analysis of 9 years experience of totally 40 cases of prehepatic portal hypertension was taken into account.All cases were diagnosed with indirect portal vein angiography and/or CTV of meso-portal vein.Surgical strategy included: 31 cases of mesocaval shunt;4 cases of splenectomy and spleno-renal vein shunt;1 case of porta-caval shunt;2 cases of esophagogastric vein excision and ligation with exploration of portal vein and inferior vena cave and another 2 cases had no-surgical option and underwent conservative treatment.Results Follow-up was performed in 39 cases ranging from 3 months to 8 years after operations.The patients without operation have not been followed up.Satisfactory outcomes were obtained from 36 cases with various portal-caval shunts, including disappearance of a syndrome of hypersplenism and no recurrence of gastrointestinal hemorrhage.On the other hand, 2 cases treated without shunt were dead within 8 months of follow-up and one of the two cases with conservative treatment was dead either.Conclusion Portovenography and/or CTV is a standard diagnosis method for pre-hepatic portal hypertension.Surgical management of portacaval shunt is a good choice for pre-hepatic portal hypertension.