We report a rare case of intrahepatic cholangiocarcinoma associated with old infestation of Schistosoma japonicum. The patient was a 76-year-old Japanese man who had lived his childhood in an endemic area of this parasite. He presented with jaundice and computed tomography showed a 4-cm, hypodense tumor in segment VIII of the liver. Microscopically, the resected mass was composed of well-differentiated adenocarcinoma cells. Fibrosis and inflammation were seen around the dilated peripheral portal veins embolized with dead S. japonicum eggs. Our search of the literature found only one other case of cholangiocarcinoma coincident with S. japonicum, suggesting that it is not a risk factor for cholangiocarcinoma, although the inflammation and fibrosis caused by the S. japonicum eggshells may predispose to carcinogenesis. However, there is no evidence supporting this hypothesis. More data are necessary to evaluate the differences in clinicopathological findings between cholangiocarcinoma concomitant with S. japonicum and the usual type of cholangiocarcinoma.