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Pancreatic hepatoid carcinoma (PHC) is a rare form of pancreatic malignancy mimicking hepatocellular carcinoma (HCC) in morphology, immunohistochemistry, and behavior. PHC usually has an aggressive clinical course and poor prognosis. Effective treatment strategies are lacking due to poor understanding and extreme rarity of such kind of malignancy.we present the case of a 75-year-old man with tumors in the liver and the tail of pancreas that were detected in a routine health check-up.Pancreatic hepatoid carcinoma with liver metastasisThe patient was treated by neoadjuvant modified-FOLFIRINOX (mFOLFIRINOX) chemotherapy and subsequent resection of both the primary pancreatic tumor and the sole liver metastasis.Pathology findings confirmed complete pathological response of the liver metastatic tumor and partial response of the primary pancreatic tumor to neoadjuvant mFOLFIRINOX. Adjuvant chemotherapy with mFOLFIRINOX was given and until now the patient has a progression-free survival of 13 months after diagnosis.PHC are often associated with early liver metastasis and a poor prognosis, surgical resection combined with neoadjuvant and adjuvant FOLFIRINOX chemotherapy is proved to be efficient in such kind of malignancy, even with liver metastasis.