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Congenital hereditary endothelial dystrophy (CHED) may be present at birth in the form of whitish cloudy corneas. Descemet stripping endothelial keratoplasty has been attempted to solve the problem but never in babies. We report a case of successful bilateral surgery in a 3-month-old baby.CHED was detected at birth in a newborn female, but surgery was deferred to increase the chance of success. At 3 months, endothelial keratoplasty was performed in the left eye without any attempt to remove the existing endothelium. As the cornea cleared up soon after the surgery, the right eye was operated 4 weeks later.Both corneas were clear 10 and 9 months after the surgery, respectively. Central thickness was 580 μm in the right eye and 530 μm in the left eye. The baby followed the light and started smiling at the lactating mother. There were no signs of graft rejection. Steroid eye drops are currently employed.Endothelial keratoplasty without endothelium removal has been successfully employed in a newborn with CHED for the first time. This procedure may be the best option for newborns with cloudy corneas.