Thirty-four patients with biliary atresia have undergone Kasai's operation in our clinic during the past 12 years. Of 21 patients who showed good bile excretion postoperatively, 8 are surviving more than 2 years later. 4 of these survivors and 2 who died of hepatic failure after operation which succeeded in achieving bile excretion, developed portal hypertension with esophageal varices. More than 30 similar patients have been reported in the literature. Esophageal varices after a successful Kasai's operation developed in one fourth of the patients who were expected to be cured. Half of these went on the hemorrhage. Portal hypertension due to biliary hepatic fibrosis could be divided into acute and chronic types. The acute type had a poor prognosis due to concomitant severe postoperative cholangitis, whereas, almost all patients with the chronic type survived, if the esophageal varices were well controlled conservatively or operatively. Transthoracic esophageal transection with paraesophagogastric devascularization seems to be the treatment of choice, and splenectomy may be added through the diaphragm only in patients with hypersplenism. Major or minor shunting procedures should be avoided because they decrease the blood flow to the liver.