A78-year-oldmanwasadmittedtoourhospitalforinvestigationofliverfunctionabnormalities.Contrast-enhancedcomputedtomography(CT)showedahigh-enhancedtumorinthelowerbileduct.Endoscopicretrogradecholangiopancreatographyshowedstenosisofthelowerbileductanddilationoftheupperbileduct.Biopsyofthetumorsuggestedsmallcellneuroendocrinecarcinoma,andsubtotalstomachpreserving pancreatoduodenectomy was performed. Abdominal CT performed 12 months after the surgeryshowedtwometastasesintheliver.Chemotherapywasstartedwithetoposide(ETP)andcarboplatin(CBDCA).Afterthechemotherapy,thelivermetastasesshrank.Thepatienthasremainedalive morethan20monthsaftersurgery.PrimaryNECofthebileductisextremelyrare,anditsprognosisis reportedtobeextremelypoor.Thechemotherapywaseffectiveinthepresentcase.Therefore,thiscase isreportedalongwithashortdiscussionoftheliterature.