Of 366 cases of portal hypertension in adult patients referred for evaluation and management in the past 15 years, the cause was not related to cirrhosis or hemochromatosis in 41. No specific cause was demonstrated for portal hypertension in four cases, which were excluded from further evaluation. Of the remaining 37 patients, 26 had a presinusoidal block characterized primarily by bleeding from esophagogastric varices, and 9 had a postsinusoidal block characterized by the rapid development of intractable ascites. In two cases an arteriovenous fistula was the cause of portal hypertension. Treatment was operative or nonoperative depending on the nature and prognosis of the basic disease. The various approaches to therapy include shunting procedures for the control of ascites or esophagogastric varices, the use of a type of portal-azygous disconnection and a direct approach to a valve or a fistula. In the absence of a rapidly fatal primary disease, portal hypertension is not a threatening problem and may be controlled with minimal mortality by appropriate surgical management.