【Abstract】 ObjectiveToinquireintodiagnosis,differentialdiagnosisandtreatmentofvirilizingandfeminizingadrenalsyndromeespecialydifferentialdiagnosisbetweenbenighandmalignantofsexhormoneproducingadrenalneoplasmaandtreatmentprinciplesofcongenitaladrenalhyperplasia(CAH). Method EightcasesofCAHandfivecasesofsexhormoneproducingadrenalneoplasmawerepresentedduring1986~1996.Theformerincluded3rarecasesof17αhydroxylasedeficiencyandothers.Thelatterincluded3casesoffeminizingadrenaltumorsand2casesofvirilizingadrenaltumors. Result Weightanddiameteroftumor,DHEA,17-ksandsexhormonelevels,appearanceofCTimaging,infiltrationandmetastasis,werecloselyrelatedtodifferentiationofbenighandmalignenttumors. Conclusion Somestandardsarenotabsolutebecauseoflimitedpractice,follow-upisveryimportant.Adrenalvirilizingandfeminizingneoplasmsweresurgicallyresectedbydifferentincision.Modifiedsubcostalincisionisrecommendedasabestchoiceforhugeadrenalmass.CorticoadrenalhormonetreatmentofCAHshouldselectdifferentkindofcorticoadrenalmedicinefordifferentkindofCAH.Treatmentofsexhormonesisnotsuitableforchildrensufferedfrom17hydroxylasedificincyuntilprepuberty.