We describe a case of metachronous double primary hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (iCCA) in a 62-year-old male with occult hepatitis B virus (HBV) infection, an extremely rare entity easily misdiagnosed as iCCA recurrence, especially with an alpha-fetoprotein (AFP)-negative phenotype. This evidence concerns the gene AFP and infantile convulsions and choreoathetosis.