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Although the frequency of hepatoblastoma is low, it is the most common primary malignant liver tumor in children. The prognosis of the disease has improved considerably in the last decades due to oncological and surgical treatment advances. Nonetheless, tumors which are diagnosed at an advanced stage still have a poor prognosis. We present the case of a 33-month-old child, diagnosed with high-risk hepatoblastoma (pulmonary metastases) in February 2014. Surgery was performed and the tumor completely removed. Afterwards, chemotherapy treatment was initiated according to a modified SIOPEL-4 protocol - the chemotherapy blocks which should have been administered before surgery were received after the operation. After finishing the chemotherapy, the thoraco-abdominal CT scan indicated a complete response to treatment. The periodic evaluation of the patient revealed the absence of local tumor recurrence, the absence of metastases, and a Lansky performance status of 80 up to now. The administration of targeted molecular therapies, liver transplant, and new chemotherapy drugs could improve the prognosis for patients with high risk hepatoblastoma in the future. Also, modifying the chemotherapy protocols could be considered an option in the achievement of this goal. [ABSTRACT FROM AUTHOR]