We report three cases of extrahepatic portal-systemic encephalopathy (EHPSE), two of which did not show portal hypertension (PH) and in the other of which precirrhotic state with chronic active hepatitis was detected at liver biopsy. Our review of the literature regarding EHPSE without PH leads us to conclude that a part of the shunts is congenitally or spontaneously formed, that PH is not always essential to shunt formation. Since the clinical symptoms most often do not manifest until middle age, cases with only mild disturbance of consciousness are not easily distinguished from dementia. Furthermore, all reported patients underwent shunt closure followed by marked improvement of clinical symptoms and uneventful postoperative course. EHPSE without PH which can be corrected by shunt closure should always be differentiated from EHPSE with PH. hepatic encephalopathy; portal-systemic shunt; portal hypertension; dementia; shunt closure Portal-systemic encephalopathy (PSE) (Sherlock et al. 1954) is now thought to occur because portal venous blood bypasses the liver and drains directly into the systemic circulation through a portal-systemic shunt (PSS). PSE is usually accompanied by portal hypertension (PH). PSE without PH has long been thought to be very rare and there are only seven cases in the English-language literature to date (Raskin et al. 1964(Raskin et al. , 1984Kozuka et al. 1975;Honda et al. 1978;Chagnon et al. 1986;Ohtomo et al. 1986;Mori et al. 1987). We encountered three patients of extrahepatic portal-systemic encephalopathy ( EHPSE ). Two did not show PH, and the other showed precirrhotic state with chronic active hepatitis at liver biopsy. We compare the findings in our three patients and review reported