Combined hepatocellular and cholangiocarcinoma (cHCC-CC) is an uncommon form of primary liver cancer having features of both hepatocellular and billiary epithelial differentiation. We report a case of cHCC-CC in a patient who was serologically positive for hepatitis B virus. A 39-year-old male was diagnosed by ultrasonography with an asymptomatic tumor in the left lobe of the liver. Based on radiologic and serologic findings of elevated serum alpha-fetoprotein level, a preoperative diagnosis of hepatocellular carcinoma was made, but differential diagnosis included focal nodular hyperplasia, because tumor was enhanced in delayed phase in Godolinium MRI scan. A final diagnosis of cHCC-CC was made after operation.