简介：Hepaticinvolvementinaggressivesystemicmastocytosis(ASM)isrelativelycommon,andthemainclinicalfeaturesofthisdiseaseincludehepatomegaly,portalhypertension,ascites,andfibrosis.CirrhosisisarareASMsymptom.WereportanASMcasethatinitiallymimickedcirrhosisbasedonclinicalandradiographicanalyses.Theportaltractwasexpandedbymononuclearinflammatorycells,andanincreaseincollagenamountwasobservedinroutinehistologicalsectionsofthebiopsiedliver.Adiagnosisofsystemicmastocytosis(SM)wasmadeafterancillarytestsformastcellsusingbonemarrowaspirates.Extensiveinvolvementoftheliverandgastrointestinaltractwasobserved.CliniciansandpathologistsneedtoconsiderASMasadiagnosisordifferentialdiagnosisinaclinicalcaseofcirrhosiswithunknownetiology.Thediagnosiscanbeconfirmedordisregardedbyimmunohistochemicalstainingandmolecularanalysis.