Patients with extrahepatic portal vein thrombosis may present from infancy through adulthood with variceal bleeding. Physiologically, such patients differ from patients with cirrhosis and variceal bleeding in that they have a normal liver and maintain good portal perfusion through hepatopedal collaterals. Complete evaluation of these patients requires identification of the bleeding site by endoscopy, definition of the anatomy by angiography, and confirmation of a normal liver by biopsy examination. Causative factors, including hypercoagulable states, should be evaluated. Therapeutic options range from noninterventive, through ablative procedures, to shunt operations. The goal should be definitive control of bleeding and return to a normal lifestyle. Distal splenorenal shunt offers the best option if technically feasible, but if no shuntable veins are patent, ablative procedures and sclerotherapy may be required. A noninterventive, noninvestigational approach is inappropriate in patients who can be offered definitive therapy. Splenectomy for hypersplenism should not be done in these patients.