Combined hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC) is a rare tumor entity. In this report, we describe a case of a young patient who developed a liver tumor in a cirrhotic liver caused by cystic fi brosis. All diagnostic fi ndings suggested that this tumor was an HCC. We performed living donor liver transplantation. Histological examination of the tumor revealed combined HCC and CC as an incidental fi nding. Two years after the transplantation, the patient is in good clinical condition and is disease-free.