Neonatal diabetes mellitus is regarded as genetic, diversified, and infrequent disorder of babies. This is developed due to mutation of specific gene. ABCC8 and KCNJ11 genes mutation are fundamental cause for the development of this. These genes encrypting the potassium network and are very much involved for seeking the monogenic diabetes like Neonatal diabetes mellitus. Neonate diabetes is classified as transient diabetes mellitus and permanent diabetes mellitus. Transient diabetes mellitus is observed at infancy stage where as Permanent diabetes mellitus is observed at 7 weeks to 26 weeks. With the help of genetic screening, this classified diabetes is discriminated. Transient diabetes is observed during infant stage and furtherly starts in later life. 22 genetic variations are very much interconnected with this type of diabetes. This article will provide a summary of current knowledge on neonatal diabetes mellitus.