Context: Budd–Chiari syndrome (BCS) is a heterogeneous group of clinical conditions ranging from acute liver failure to completely asymptomatic patient. Patients with BCS nonresponsive to medical treatment or who are not candidates for angioplasty/stenting are routinely treated with transjugular intrahepatic portosystemic shunt (TIPS) to improve portal venous outflow. Aims: This retrospective study aims at determining the short and long-term outcomes of TIPS in BCS patients. Subjects and Methods: This retrospective study included 118 patients with primary BCS who underwent TIPS from 2005 to 2011. They were classified according to Child-Pugh, model for end-stage liver disease (MELD), Rotterdam and BCS-TIPS scores. Short-term outcome of TIPS (<1 year) was determined and included immediate postprocedure complications, effect on clinical status, liver profile, and stent patency. Long-term outcome (1–5 years) was determined and included stent patency rate, time, and pattern of management of stent occlusion and survival rates at 1, 3, and 5 years. Statistical Analysis Used: SPSS statistical package version 16. Paired t-test was used to compare pre and post-TIPS MELD and Child-Pugh scores. Chi-square test and Kaplan–Meier survival curve were used to examine the association between short- and long-term occlusion, liver function and survival outcomes. P < 0.05 was considered statistically significant. Results: By the 1st year, there was a significant decline in the percentage of patients having ascites and abdominal pain, increase in mean serum albumin from 3.42–4.02 g/dl and reduction in bilirubin levels from 2.57–1.35 mg/dl (P < 0.001). The rate of stent occlusion by the 1st year was 27.4%. During long-term follow-up, there was a marked improvement in clinical status, liver profile, Child–Pugh, and MELD scores (P < 0.001). The 1- and 5-year shunt patency rates were 72.6% and 78.7%, respectively. The main management of post-TIPS occlusion was angioplasty without stenting. The 1-, 3-, and 5-year survival rates were 95.8%, 93.2%, and 91.5%, respectively. There was a significant relation between survival and post-TIPS MELD score (P = 0.009). Conclusions: TIPS is an effective treatment modality for BCS and has a long-term survival benefit with satisfactory shunt patency and patient survival.