摘要Despite hepatocellular carcinoma's position as the second most common pediatric liver tumor, it is a rare tumor in children warranting international collaboration to improve outcomes. Few cases diagnosed in earlier stages, when confined to the liver and responding to systemic treatment or with resectable metastases, may be cured by complete resection and/or orthotopic transplantation. Complete resection is the only chance for cure; therefore, all attempts should be made to make these options available. Despite modest progress in locoregional treatments, these serve in most cases as palliative treatment or as a bridge to definitive treatment at best. Currently used systemic treatments have response rates below 50％. Five-year survival in advanced stages is below 30％. The international Paediatric Hepatic International Tumour Trial trial is evaluating novel systemic treatments in pediatric hepatocellular carcinoma. Patients suffering from these tumors likely benefit from targeted treatment based on molecular aberrations corresponding with tumor subtype.