Fibrolamellar carcinoma (FL-HCC) is an unusual and little-known form of hepatocellular carcinoma (HCC). It is quite uncommon. In the remarkable absence of underlying liver illnesses, it primarily affects the young population in humans, specifically children and young adults. With metastases typically discovered in the lung and peritoneum in addition to the regional lymph nodes, FL-HCC has unique clinicopathologic characteristics. Despite being thought to have a slow progression at first, survival results have been revised recently, suggesting that the tumor is hiding much of the biogenesis. The prognosis, anatomy, and molecular mechanisms of this uncommon tumor are discussed in this review.