An overview of the circulation of the liver and of the pathogenesis of hepatic encephalopathy as a result of portal vascular anomalies is given. Clinical signs associated with portal systemic shunts are described on the basis of 16 cases, 14 dogs and 2 cats. These animals ranged in age at the time of presentation from 4 months to 7 years. The predominant abnormality observed were central nervous signs, which differed in severity. The different techniques of contrast angiography allowing demonstration of a portal systemic shunt are presented along with a discussion of the pros and cons of each. Additionally the significance of making portal venous pressure measurements prior to each angiography is also explained. In most cases mesenteric portography was chosen. Based on their location the anomalies could be categorized as intrahepatic (4 dogs) or extrahepatic (10 dogs, 2 cats). In both groups breeds of various size are represented. The extrahepatic shunts could be further described as portal-caval (n = 5), portal-phrenic (n = 4) and portal-azygos (n = 3). In five of the older animals angiography showed in addition some hepatic perfusion by the portal vein. Laboratory evaluation revealed increased resting blood ammonia concentrations (greater than 200-912 micrograms/100 ml) in all animals. Seven dogs had definitely subnormal BUN concentrations (less than 10 mg%) and ten dogs low total plasma protein levels (less than 5.4 g%). Free amino acids (24) were determined in four dogs and a lowered hepatic encephalopathy index (less than 1.64) found. Medical palliative therapy to control the clinical signs is discussed. The only effective long term therapy is, however, surgery. The shunt vessel is narrowed so that a greater volume of portal blood reaches the liver. Experience gained from the surgical therapy of 14 animals is presented. Ten of these survived well without requiring further therapy at a later time. Finally the etiology, prognosis, and differential diagnosis are summarized.