The article focuses on the improvement of metabolic control and neurologic outcome for neonatal patients based on the result of genetic confirmation of a sulfonylurea-responsive form of diabetes. Topics include the role of mutations on the ATP-sensitive K (KATP) in diabetes, the effect of sulfonylureas on KATPchannel function and the use of glyburide in neonatal patients. The case of neonatal diabetes treated with sulfonylureas is presented.