Hepatoblastoma (HB) is the most prevalent malignant liver tumor in pediatric populations, primarily affecting children under five years of age. Despite its rarity, HB accounts for the majority of childhood liver cancers and poses significant clinical challenges, particularly in high-risk and metastatic cases. The pathogenesis of HB is closely linked to genetic and epigenetic alterations, notably mutations in the CTNNB1 gene and activation of the Wnt/β-catenin signaling pathway. Syndromic associations such as Beckwith–Wiedemann syndrome and familial adenomatous polyposis further underscore its genetic predisposition. Diagnostic strategies include serum alpha-fetoprotein (AFP) quantification, immunohistochemistry, and advanced imaging techniques, with staging guided by the PRETEXT system. Standard treatment involves cisplatin-based chemotherapy, surgical resection, and liver transplantation for unresectable tumors. However, chemoresistance and recurrence remain major obstacles.