BackgroundNoncompactionofventricularmyocardium(NVM)isararetypeofprimarycardiomyopathy.Thediseaseiscausedbythedisorderinthedensificationofthemyocardiumintheearlystageoftheembryoprocess.Themorphologicalcharacteristicsareprojectingtrabeculationintheventricleandthedeeptrabecularspaceinterlinkedwithventricularchamber.Inrecentyears,manystudieshavefoundthattheleftventriculargrowthassociatedgeneticmutationiscloselyrelatedtotheoccurrenceofNVM.Themostclinicalmanifestationssuchasheartfailure,thromboembolismandarrhythmiaarespecific.EchocardiographyisthemostcommonlyusedtechniqueforthediagnosisofNVM.Cardiaccomputedtomography(CT)scan,cardiacmagneticresonanceimagingandleftventricularangiographyareotherimportanttechniquesforitsdiagnosis.TheNVMpatientshavealongcourseofdisease,poorprognosisandahighrateofmisdiagnosis.Thisarticlereviewstheresearchprogressintheaspectsofepidemiologicalcharacteristics,geneticcharacteristics,clinicalmanifestations,pathophysiology,diagnosis,treatmentandsoon,inordertoprovidethebasisforthediagnosisandtreatmentofNVM.