The prevalence of portal hypertension and its complications is increasing among patients with hemophilia and cirrhosis. The authors evaluated the safety and efficacy of transjugular intrahepatic postosystemic shunt (TIPS) placement in this population. A retrospective analysis was performed of adult patients who underwent TIPS placement at a single center. Four patients with hemophilia and cirrhosis were identified. Outcome measures included technical success and complications, recurrent gastrointestinal hemorrhage, shunt patency, hepatic encephalopathy, ascites control, and mortality. With periprocedural factor VIII supplementation, TIPS were placed in all patients without complications and with improvement in portal hypertension. Outcomes after TIPS placement appear to be comparable to those in patients without hemophilia.