Congenital hereditary endothelial dystrophy (CHED), presents in infancy or early childhood with bilateral clouding of corneas. This condition has previously been managed surgically with penetrating keratoplasty (PKP). Performing PKP in pediatric patients has its own set of difficulties. More recently, there has been growing interest in treating this condition with Descemet's stripping automated endothelial keratoplasty (DSAEK). The purpose of this study is to report our experience of successfully performing DSAEK in a child with CHED.