A 53-year-old man was admitted to our hospital with a liver mass. CT and MR images showed the presence of a multilobulated mass with hemorrhagic and necrotic foci in the left hepatic lobe. In addition, thrombosis of the left portal vein was noted. The patient underwent surgery for total removal of the mass. A pathological examination revealed the presence of an intrahepatic sarcomatoid cholangiocarcinoma with portal vein thrombosis. The patient underwent a left hepatectomy and chemotherapy. However, the disease recurred with metastases after five months. The patient underwent surgery and chemotherapy for the recurred tumor.