Abstract： Objective Enhance the identification and diagnosis of neonatal diabetes mellitus(NDM).Methods Analyzed three cases of NDM,examining their clinical features,gene results,treatment outcomes and monitore prognosis.Results Three cases successfully transitioned from insulin therapy to glibenclamide oral therapy due to KATP channel gene mutation.Two children were still taking oral glibenclamide,considered to have permanent neonatal diabetes mellitus(PNDM);one child discontinued the drug after six months of treatment and was considered to have transient neonatal diabetes mellitus(TNDM).No adverse reactions were observed in any of the cases.Conclusion Children with NDM caused by KATP channel mutation can transition from insulin to glibenclamide therapy.Early detection of the genetic mutation and administration of glibenclamide can reduce treatment costs,improve compliance,and enhance neurological prognosis.