Congenital portosystemic shunts (PPS) are abnormal vascular communications that allow blood from the intestine to bypass the liver, and are classified as intrahepatic or extrahepatic. Clinical signs are generally related to the nervous, gastrointestinal or urinary systems, and are often vague. In addition, changes present on routine blood analysis are often mild and non-specific. For this reason, alternative tests are required for a diagnosis. Diagnostic tests include serum bile-acid concentrations, ammonia tolerance test, portography, ultrasonography and/or scintigraphy. Medical therapy involves reducing absorption of encephalopathic toxins from the gastrointestinal tract and may prolong survival. Surgical therapy is aimed at attenuation of the shunting vessel and provides improved survival rates. The traditional approach has been complete or partial ligation of the shunt. More recent approaches have involved slow, progressive attenuation with ameroid constrictors or cellophane banding. Overall, prognosis following surgical therapy is good in dogs and fair in cats.