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Extrahepatic tissue is liver parenchyma that develops in other organs but retains the general morphology and/or functionality of the hepatocytes within the mother liver, including the potential for neoplastic transformation. The conversion of an ectopic liver to a malignant tissue is an extremely rare finding.We present a 30-year-old male with no past medical history who presented with altered mental status and upon further inquiring admitted to progressive abdominal discomfort, fullness, early satiety, night sweats and weight loss. Contrast-enhanced CT imaging of his abdomen and pelvis demonstrated a large intra-abdominal mass with significant mass effect on the liver. Subsequently, patient underwent a CT-guided biopsy of the intra-abdominal mass and histological features of the specimen were consistent with fibrolamellar hepatocellular carcinoma.Here, we report a rare case of metastatic fibrolamellar hepatocellular carcinoma originating in a focus of extrahepatic liver tissue located in the pancreas. The tumor was found incidentally on imaging and was associated with liver synthetic dysfunction but no elevation of alpha-fetoprotein levels carcinoma in a young patient without any prior medical history. This case is unique in that it highlights a more severe outcome of this rare tumor type and underscores the need for accurate and timely diagnosis and treatment. It discusses the treatment with bevacizumab and atezolizumab. While prior case reports typically describe surgical resection of the tumor given its limited location to the ectopic foci, our patient presented with extensive metastatic disease and was treated with immunotherapy and anti-angiogenic therapy followed by chemotherapy which ultimately did not slow the progression of his disease.