Purpose This study was performed to evaluate the safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of patients affected by Budd-Chiari syndrome (BCS). Materials and methods From January 1999 to December 2006, 15 patients (seven male and eight female subjects, age range 7–52 years) with BCS uncontrolled by medical therapy were treated with TIPS placement. In seven cases BCS was idiopathic, in four it was caused by myeloproliferative disorders and in four by other disorders. One patient also had portal vein thrombosis. In 5/15 cases TIPS was created through a transcaval approach. Eight patients (53.4%) received a bare stent, and seven (46.6%) received a stent graft. The follow-up lasted a median of 29.4 (range 3.2-68) months. Results Technical success was achieved in all patients without major complications. TIPS was very effective in decreasing the portosystemic pressure gradient from 26.2±5.8 to 10±6.2 mmHg. All patients but two were alive at the time of writing. Acute leukaemia was the cause of the single early death and was unrelated to the procedure. The patient with portal vein thrombosis underwent thrombolysis before TIPS, but the vein occluded again after 3 weeks, and the patient died 6 months later. The other patients showed significant improvements in liver function, ascites and symptoms related to portal hypertension. Primary patency was 53.3%, and primary assisted patency was 93.3%. No patient required or was scheduled for liver transplantation. Conclusions TIPS is an effective and safe treatment for BCS and may be considered a valuable alternative to traditional surgical portosystemic shunting or liver transplantation.