We describe the case of a 15-year-old boy with a history of Fontan operation and multiple intrahepatic tumors. Computed tomography showed multiple hepatic nodules with arterial enhancement. Because hepatocellular carcinoma (HCC) was not detected on biopsies and tumor markers were normal, progress was monitored on imaging. One hepatic tumor increased greatly in size during follow up. At 15 years of age, tumor markers rose rapidly, and he had upper abdominal swelling. Therefore, transarterial embolization (TAE) was performed for the largest tumor, suspected to be a HCC due to cardiac cirrhosis. This tumor had not increased at follow up 4 months later. The patient died from hepatic failure at the age of 17 years, and HCC was diagnosed at autopsy. Although pediatric HCC is rare, its incidence is likely to increase. TAE, with or without anticancer agents, is a therapeutic option for unresectable pediatric HCC, as it is for adult HCC.

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