Patients with Budd-Chiari syndrome may require therapy with portal decompressive surgery. Transjugular intrahepatic portosystemic shunt has recently emerged as a new treatment alternative to surgical shunting, but reported literature is scarce and its long-term outcome is to be evaluated. The aim of our study was to present two patients with Budd-Chiari syndrome treated with transjugular intrahepatic portosystemic shunt. Two female patients with acute and subacute Budd-Chiari syndrome respectively, uncontrolled by medical therapy, were referred for transjugular intrahepatic portosystemic shunting. Both patients had refractory ascites, while one had hepatic insufficiency. Hepatic vein thrombosis was without definitive etiology in one patient and due to essential thrombocytosis in the other. Transjugular intrahepatic portosystemic shunt was successfully created in both patients; in one a transcaval approach was performed. Bare stents were used in one, while in the other a polytetrafluoroethylene-covered stent-graft. Technical success was 100% with no complications. Ascites resolved in both patients and liver function improved. The first patient remains asymptomatic with good shunt patency 6 months after intervention. The second patient is symptom-free two years after the shunt creation, despite stent occlusion. She remains asymptomatic due to hepatic vein collateral development. In patients with acute or subacute Budd-Chiari syndrome uncontrolled by medical therapy, transjugular intrahepatic portosystemic shunt is highly effective with no complications and an associated mid-term clinical success.