OBJECTIVE: To investigate the clinical characteristics, treatment options and efficacy of prehepatic portal hypertension. METHODS: The clinical data of 46 cases of prehepatic portal hypertension, including 2 patients with Abernethy malformation, between January 2000 and May 2009 at the Second Artillery General Hospital and Peking Union Medical College Hospital were retrospectively analyzed. All patients were diagnosed with prehepatic portal hypertension based on indirect portal venography, CT angiography, and/or ultrasound findings. There were 23 cases of superior mesenteric vein-inferior vena cava shunt; 8 cases of splenectomy and splenic vein-renal vein shunt; 1 case of portal vein-inferior vena cava shunt; 2 cases of umbilical vein-internal jugular vein shunt; 3 cases of portal vein dissection; 2 cases of splenectomy and portal vein dissection; 1 case of temporary sigmoid colon fistula with fistula closure after 6 months; 1 case of most of small bowel resection; 4 cases of thrombolysis via femoral artery cannula No surgery was performed in 2 cases, and only liver protection and symptomatic treatment were given. Results: 45 patients were followed up from 2 months to 5 years, with a mean of 23.4 months, and 1 case was lost without surgery. In 34 patients treated with shunt surgery, the symptoms of hypersplenism disappeared after surgery, and no further upper gastrointestinal bleeding occurred; one case of superior mesenteric vein-inferior vena cava shunt was performed for rebleeding 13 months after shunt surgery; one case of death due to rebleeding 8 months after shunt surgery; one case of death due to intestinal necrosis 40 days after thrombolytic treatment; one case of black stool reappeared 4 months after discharge from the hospital without surgery and was treated conservatively improved. Conclusion: The treatment of prehepatic portal hypertension is based on reducing portal vein pressure, and various shunt procedures and thrombolysis of superior mesenteric artery and/or splenic artery are safe and effective, but they need to be performed according to the individual.