Attempting to elucidate the best way to treat adult patients with extrahepatic portal vein occlusive thrombosis, the experience with six such patients is analyzed. Portal vein thrombosis rarely occurs in adults but when it does, it frequently results in portal hypertension with its concomitant complications. Five patients had bleeding esophageal varices, two had massive refractory ascites. Their conventional liver function tests were not greatly altered. Thrombocytosis and history of thrombophlebitis were encountered in over half of the patients. Splenoportography is the best method to confirm the diagnosis preoperatively. In none of the six patients a decompressive portal systemic shunt could be performed although, it was attempted in five. Three patients survived. It appears that nonoperative management is the treatment of choice in these patients who can remain well for several years, however, when bleeding recurs gastric devascularization or even resective procedures are acceptable therapeutic alternatives.