Objective To explore the clinical presentation and diagnosis and treatment of prehepatic portal hypertension （PPH） and discuss its surgical strategies. Methods Forty-six cases of PPH treated in the 2nd Artillery General Hospital and Peking Union Medical College Hospital from January 2000 to May 2009 were analyzed retrospectively, including 2 cases of Abernethy abnormality. All patients were evaluated by indirect portal vein angiography, CT angiography and (or) portal duplex system Doppler ultrasonography before treament. Surgical strategies included: 23 cases with meso-caval shunt, 8 cases with splenectomy and spleno-renal vein shunt, 1 case with porta-caval shunt, 2 cases with paraumbilical vein-jugular vein shunt, 3 cases with portal azygous disconnection, 1 cases with splenectomy and portal azygous disconnection, 1 case with sigmoidostomy and closed the fistula of sigmoid six months later, 1 case with resection of part of small intestine due to acute extensive thrombosis of portal vein system, 4 cases with selective superior mesenteric artery and (or) splenic artery thrombolytic infusion therapy, 2 cases remained no-surgical option and underwent conservative treatment. Results Forty-four patients were followed-up from 2 months to 5 years， average of 23.4 months, one patient without surgical treatment was lost. Satisfactory outcomes were obtained in 34 patients with various shunts, which expressed as a release of hypersplenism and gastrointestinal hemorrhage. Two cases were treated with meso-caval shunt because of rehemorrhage in month 13 and 24 and one died in month 8 after disconnection, one died on day 40 after thrombolytic therapy due to putrescence of intestines, one who remained no-surgical option underwent hemorrhage 4 months later, and then went well by conservative treatment. Conclusion The key of treatment of PPH is to reduce the pressure of hepatic portal vein. Surgical managements of shunt and selective superior mesenteric artery and (or) splenic artery thrombolytic infusion therapy are safe and effective, but individual treatment strategy should be performed.