A 23-year-old woman with liver cirrhosis secondary to primary sclerosing cholangitis was referred to us for the treatment of recurrent bleeding from esophageal varices that had been refractory to endoscopic sclerotherapy. Her portal vein was occluded, associated with cavernous transformation. A transjugular intrahepatic portosystemic shunt (TIPS) was performed after a preprocedural three-dimensional computed tomographic angiography evaluation to determine feasibility. The portal vein system was recanalized and portal blood flow increased markedly after TIPS. Esophageal varices disappeared 3 weeks after TIPS. Re-bleeding and hepatic encephalopathy were absent for 3 years after the procedure. We conclude that with adequate preprocedural evaluation, TIPS can be performed safely even in patients with portal vein occlusion associated with cavernous transformation.