An increasing number of patients with Budd-Chiari syndrome (BCS) have undergone transjugular intrahepatic portosystemic shunt (TIPS). However, the critical role of TIPS in the treatment of BCS has not been systematically reviewed. The authors identified all relevant literatures via the PubMed, EMBASE and Cochrane library databases. Overall, 160 papers from 29 countries reported the application of TIPS for BCS. The number of publications was increased over time, but the level of evidence in this field was low. Common indications for TIPS in BCS patients included refractory ascites, recurrent variceal bleeding, diffuse hepatic vein thrombosis and progressive liver failure. Successful TIPS insertion could improve the hemodynamic and clinical parameters. TIPS procedure-related complications were not infrequent (range: 0-56%), but procedure-related death was rare. Shunt dysfunction rate appeared to be higher (range: 18-100%). Compared with bare stents, covered stents could significantly decrease the rate of shunt dysfunction. Hepatic encephalopathy rate after TIPS was relatively low (range: 0-25%). Short- and long-term prognosis of BCS-TIPS patients was excellent with 1-year cumulative survival rate of 80-100% and 5-year cumulative survival rate of 74-78%. In conclusions, existing literatures supported the feasibility, safety and efficacy of TIPS in the treatment of BCS. Prospective cohort studies or randomized controlled trials were difficult due to the rarity of BCS, but might be very necessary to precisely identify the timing of transition from medical therapy and/or percutaneous recanalization to TIPS insertion and the real candidates in whom early TIPS should be promptly employed with no need of any prior therapy.