A 62-year-old man was referred to our hospital for examination of a liver tumor in the hepatic left lobe. He had diabetes mellitus and prior hepatitis B virus infection. Laboratory test results for carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA 19-9) were unremarkable. Computed tomography (CT) scans of the abdomen and gadoxetic acid-enhanced magnetic resonance imaging (EOB-MRI) revealed a hypovascularized tumor, measuring 30 mm in hepatic segment 2 (S2), and no enlarged regional lymph nodes. A liver biopsy was performed to analyze the tumor. A histopathological examination showed adenocarcinoma. On immunohistochemistry, the carcinoma cells were positive for cytokeratin 7 (CK7), CA 19-9, and EMA, and negative for CK20, α-fetoprotein, and thyroid transcription factor-1 (TTF-1). The patient was diagnosed with a mass forming (MF) type of ICC. A left lateral sectionectomy, with regional lymph node dissection along the proper, left and middle hepatic arteries and the upper branch of the left gastric artery, was performed. Histopathological examination showed moderately differentiated adenocarcinoma in hepatic S2, with one lymph node metastasis around the portal vein in the hepatoduodenal ligament and the minor branch of the portal vein invasion in the main tumor; s0, n1, vp1, vv0, va0, and p0. There were two intrahepatic metastases in the same S2 around the main tumor. According to the eighth edition of the TNM staging system of the Union for International Cancer Control [], the pathological stage of the ICC was pT2pN1M0pStageIIIB. The postoperative course was uneventful, and the patient was discharged on the tenth postoperative day. Although the adjuvant chemotherapy was recommended to him because there was a high possibility of recurrence of the carcinoma, he refused to undergo it. Twelve months after surgery, liver lesions in S4/S8 and S7 were detected on CT scans. No other liver lesions were found using EOB-MRI. On positron emission tomography-computed tomography (PET-CT), abnormal fluorodeoxyglucose (FDG) uptake was observed only in hepatic tumors, and extrahepatic lesions were not detected. Neither CEA nor CA 19-9 was elevated. The patient wanted a second opinion on treatment other than surgery and chemotherapy. After observation for 3 months, the size of two recurrent liver tumors was slightly larger compared with that observed 3 months ago. However, without developing any other lesions, he underwent a partial hepatectomy for each lesion. A pathological examination of both resected tumors revealed moderately differentiated adenocarcinoma in the center of the tumors, which was similar to that of previous ICC. At the margin of the tumors, poorly differentiated adenocarcinoma was detected. On immunohistochemistry, the carcinoma cells were positive for CK 7 and negative for CK 20 and TTF-1. Histopathological features were similar to those of the previous ICC; therefore, the patient was diagnosed with recurrence of ICC. He was discharged on the seventh postoperative day. Although the adjuvant chemotherapy was repeatedly recommended to him, he refused to undergo the therapy after the repeat hepatectomy. Four years and four months after the repeat hepatectomy, CT scans showed multiple nodes in S4 and S10 of the left lung and in S1 of the right lung. On PET-CT, FDG uptake was observed only in S4 of the left lung. After observation for 3 months, the size and number of tumors did not change. Therefore, wedge resection of the left upper lobe and sectionectomy of S10 of the left lung were performed. The histopathological findings of the resected lung nodules were compatible with metastatic ICC. On immunohistochemistry, the carcinoma cells were positive for CK 7 and negative for CK 20 and TTF-1. The nodule noted in S1 of the right lung was too small to be diagnosed for metastasis; therefore, it was not resected. After pulmonary resection, He was treated with gemcitabine (1000 mg/m2) and cisplatin (25 mg/m2) which were infused on day 1 and day 8. This regimen was repeated at 21-day intervals for 6 months. After chemotherapy, the size of the nodule in S1 of the right lung increased gradually. One year and ten months after the pulmonary resection, we confirmed that there were no other metastatic lesions and we performed a wedge resection of S1 of the right lung. Histopathological findings were compatible with metastatic ICC. On immunohistochemistry, the carcinoma cells were positive for CK 7 and negative for CK 20 and TTF-1. The patient is alive without evidence of disease 8 years after the initial surgery and 8 months after the last pulmonary resection.