A 34-year-old man presented with gastrointestinal bleeding related to esophageal varices in 2002. The patient's varices were controlled with endoscopic ligation. Evaluation of the cause of the varices revealed extrahepatic portal vein thrombosis with normal findings on liver biopsy. During the follow-up period, he was found to have an increased alkaline phosphatase level; no other laboratory abnormalities of the liver were found, including the serum bilirubin level. The intrahepatic biliary ductal dilation was documented by computed tomographic cholangiography (Figure 1). A biliary stent was placed endoscopically with normalization of the alkaline phosphatase level. Computed tomographic portography demonstrated a patent intrahepatic portal vein and a patent confluence of the splenic and superior mesenteric veins, but with an occluded portal vein between the confluence and the intrahepatic portal vein with extensive venous collaterals.