Developmental screenings and neurology referrals were left to the discretion of the patient's primary care physician as the pathogenic variant c.679G>A (p.Glu227Lys) in KCNJ11 is primarily associated with maturity-onset diabetes of the young (MODY) and is low risk for developmental delay, epilepsy, and neonatal diabetes (DEND) syndrome. Here, KCNJ11 is linked to DEND syndrome.