Fibrolamellar hepatocellular carcinoma (FHCC) is a rare primary malignancy of the liver for which data remain limited. This tumor is more often diagnosed in younger patient populations in the absence of underlying cirrhosis and hepatitis. These lesions can be diagnosed on computed tomography scan or magnetic resonance imaging with common findings including central calcifications, a central stellate scar, and radiating fibrotic bands. Laboratory markers have not proved useful for diagnosis; however, pathologic analysis can be implemented to aid in diagnosis with findings including ample granular eosinophilic cytoplasm, nuclei with open chromatin and prominent macronuclei, hyaline and pale bodies, and dense lamellar fibrosis that divides the cells into cords or trabeculae. FHCC demonstrates aggressive malignant potential with nodal spread. Treatment patterns have remained mainly surgical; however, systemic therapies have been implemented and are under further investigation with clinical trials. Locoregional therapies and radiation therapies have been trialed sparingly. In this focused review, we discuss the most up-to-date perspective on epidemiology, clinical presentation, diagnostic approach, differential diagnosis, treatment regimens, prognosis, and future directions of FHCC.