Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare and distinct subtype of primary liver cancer that predominantly affects adolescents and young adults without underlying liver disease. Unlike conventional hepatocellular carcinoma (HCC), FLC is characterized by unique histological features, including large polygonal tumor cells with eosinophilic cytoplasm and prominent fibrous lamellae. A hallmark genetic alteration in FLC is the DNAJB1-PRKACA fusion gene, present in approximately 80% of cases, which drives oncogenesis through aberrant protein kinase A signaling. Despite its distinct biology, FLC lacks effective systemic therapies and is often resistant to conventional chemotherapy, partly due to the high expression of multidrug resistance genes. Surgical resection remains the mainstay of treatment, although recurrence rates are high. Recent advances in patient-derived xenograft and organoid models have provided new insights into tumor biology, including the role of cancer stem cells and potential therapeutic targets.