Objective To study the diagnosis and treatment of portal hypertension in secondary biliary cirrhosis(PHSBC). MethodsTwenty-five cases of PHSBC within recent 16 years in our hospital were analyzed. Their clinical, pathological and follow-up data were reviewed retrospectively. They were divided into 4 groups according to their primary diseases:13 patients with hepatolithiasis, 6 with postoperative stricture of biliary duct, 4 with malignancy of biliary duct and 2 with others.Results All patients were diagnosed clinically, and 4 were further pathologically confirmed. Eight cases were treated conservatively, while the remaining 17 underwent operations according to their primary diseases, and one combined with splenectomy and esophagogastric devascularization. The rate of discharge with improvement by surgical and non-surgical treatment was 64.7% and 37.5%, and hospital mortality was 17.6% and 12.5% respectively. Conclusion The diagnosis of PHSBC mainly depends on its characteristic clinical manifestations. The early surgical resolution of bile duct obstruction is the key to good prognosis. If complicated with rupture and hemorrhage of oesophagofundal varices, the surgical procedure should be considered carefully.