Objective To analyze the prognosis and indications of transjugular intrahepatic portosystemic shunt (TIPS) in patients with Budd-Chiari syndrome (BCS). MethodsPatients with primary BCS who received TIPS in the Department of Gastroenterology, West China Hospital of Sichuan University between February 2009 and February 2020 were retrospectively reviewed. The medical history, preoperative imaging, surgical records, and postoperative outpatient follow-up medical records were recorded. The laboratory indexes before and after operation were compared, and the cumulative free from hepatic encephalopathy rate, stent patency rate, and cumulative survive rate were calculated. Cox proportional hazards model was used to analyze the independent risk factors of hepatic encephalopathy, shunt dysfunction and death. Results A total of 48 patients were included. The main indications for TIPS included variceal bleeding (16 cases), refractory ascites (24 cases), and diffuse obstruction of hepatic vein with acute liver function impairment (8 cases). The cumulative 1 year, 2 years and 3 years of free from hepatic encephalopathy rates were 92.3%, 89.2% and 85.3%, respectively. The stent patency rates were 89.7%, 72.2% and 54.8% at postoperative 1 year, 3 years and 5 years, respectively. The cumulative survival rates were 86.0%, 79.5% and 71.4% at postoperative 1 year, 3 years and 5 years, respectively. Conclusion TIPS can achieve good efficacy in patients with BCS, and most patients receive TIPS for portal hypertension complications rather than acute liver function impairment.