Hepatocellular carcinoma (HCC) is considered the leading cause of primary liver malignancy, however, a tumor arising from the biliary tree should be always kept among the differential diagnosis. Cholangiocarcinoma (CCA) is a group of primary liver malignancies that represents 10-20% of all primary liver masses being perihilar cholangiocarcinoma the most common and intrahepatic the less common subtype. Important biomarkers such as serum alpha-fetoprotein (AFP) are commonly measured. An elevated AFP > 400 ng/mL is very specific for HCC. On the contrary, AFP levels rarely exceed 20 ng/mL in patients with intrahepatic cholangiocarcinoma. We report a case of a patient with a markedly elevated serum AFP who was found to have ICC.

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