We describe a case of intrahepatic cholangiocarcinoma (ICC) in a 50-year-old man. A well-defined, hypoechoic tumor, 3.5 cm in greatest diameter, was detected in the left medial segment of the liver with ultrasonography. Celiac angiography showed staining at the same location. Computed tomography revealed lymph node swelling around the head of the pancreas. On October 10, 1993, the patient underwent partial hepatectomy with pancreatoduodenectomy and lymph node dissection around the hepatoduodenal ligament and along the common hepatic artery. Postoperative histopathological examination showed a moderately differentiated tubular adenocarcinoma which had metastasized to the dissected lymph nodes at the posterior surface of the head of the pancreas and at the root of the middle colic artery. Eight years after surgery, the patient is alive and well with no sign of recurrence. Immunohistochemical staining showed ductal-type mucin core protein-1 expression in the tumor, which indicates more favorable survival after surgery. Patients with ICC and lymph node metastasis are considered to have poor prognosis; however, further study of the characteristics of ICC with lymph node metastasis is needed.