Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rarevariant of hepatocellular carcinoma. It is commonly reportedin the younger population with no underlying chronic liverdisease and free of viral Hepatitis B and C. Local recurrenceand distant metastasis are common despite betterprognosis compared to conventional hepatocellularcarcinoma. Complete surgical resection is associated withhigher median survival and is the mainstay treatment optionfor localized FL-HCC. Multi-modality therapies such as TACEcan be used to downstage upfront unresectable FL-HCC.Complete response with GEMOX chemotherapy has beenreported in advanced metastatic FL-HCC and should beconsidered in upfront unresectable or metastatic disease.We present a case of biopsied proven relapse FL-HCC witholigo- left lung metastasis who successfully underwent a leftlung lobectomy after neo-adjuvant GEMOX chemotherapy,and is disease free at 24 months follow up.