Fibrolamellar (FL) hepatocellular carcinoma (HCC) is a distinctive form of primary HCC that occurs principally in children and young adults. Although liver transplantation is not contraindicated for FL-HCC, noncirrhotic patients with large HCC tumors (including FL-HCCs) are not prioritized. Although hepatic resection is considered to be the primary treatment for FL-HCC, living donor liver transplantation is evolving into a potentially better alternative. Herein we have reported successful "preemptive" living donor liver transplantation for presumed recurrence of FL-HCC after an extended right hepatectomy with resection and synthetic graft replacement of the inferior vena cava.