We present a case of long-term survival in a patient that involved intrahepatic cholangiocarcinoma that metastasized to the paraaortic lymph nodes. A 62-year-old man underwent extended left hepatic lobectomy with caudate lobe resection, extrahepatic bile duct resection, portal vein resection and reconstruction, and middle hepatic vein resection and reconstruction with lymph node dissection for a liver tumor that was located in the caudate lobe. Histological examination of the resected specimen revealed moderately differentiated adenocarcinoma compatible with cholangiocarcinoma, and lymph node metastases were found in the area of the hepatoduodenal ligament and the paraaortic region. After surgical resection, recurrence was detected twice in the lymph nodes at the site of the left supraclavicular region. These recurrent tumors were completely eliminated by systemic chemotherapy with cisplatin or mitomycin C. The patient is now doing well 6 years and 5 months after surgical treatment. In this case, there was only one tumor, and the preoperative serum carbohydrate antigen 19-9 level was normal. In addition, heterozygosity was retained at the loci on chromosome 8p. These findings suggested that tumor in the present case was less aggressive, despite the nodal spread. The extensive surgical approach may have contributed to the long-term survival of this patient, while the recurrent tumor was sensitive to antitumoral agents.