Regional portal hypertension (RPH) results from splenic vein thrombosis/occlusion, with emergence of gastric varices and severe upper gastrointestinal bleeding. Its diagnosis and management are summarized below. We reviewed our experience in 16 consecutive patients with RPH at Chinese PLA General Hospital between 2001 and 2004. The clinical manifestations, diagnostic methods and therapeutic modalities were analyzed retrospectively. Among the 16 patients with RPH, 12 had co-existing pancreatic diseases and 4 were of obscure origin. The main clinical findings were splenomegaly in 16 (100%), epigastric pain (mainly left upper abdomen) in 10 (63%), gastrointestinal bleeding in 7 (44%) and abdominal mass in 3 (19%). All had normal liver function tests. The main diagnostic methods used were ultrasound (US), computerized tomography (CT) and endoscopy. Splenic vein thrombosis could be identified by color Doppler ultrasound (7/7), enhanced CT could show its enlarged and tortuous short gastric vein, gastroepiploic vein, and collateral vessels around the splenic hilum (16/16). Isolated gastric varices (4/5) were mainly shown by esophagogastroscopy. Splenectomy was effective in controlling the variceal bleeding, but portal vein thrombosis occurred in 1 case. Endoscopic sclerotherapy had been carried out repeatedly in 2 cases, but was less effective (1/2, 50%). Precise diagnosis of RPH can be achieved by clinical manifestations, US and CT. Therapeutic options should be individualized basing on the underlying diseases, however, splenectomy is more preferable for patients with bleeding varices, and endoscopic sclerotherapy can be tried.