The purpose of this study was to report our experience of an attempted Descemet's stripping automated endothelial keratoplasty (DSAEK) in a patient with congenital hereditary endothelial dystrophy (CHED). A 7-year-old boy presented with the complaints of decreased vision in his right eye. The left eye had undergone penetrating keratoplasty at the age of 5 years. He was diagnosed as a case of CHED, and a DSAEK was planned for him. Due to technical difficulties during Descemet's membrane scoring and stripping and poor visualization, the procedure had to be abandoned and converted to a full thickness penetrating keratoplasty. To the best of our knowledge, this is the first reported case of an attempted DSAEK in a patient with CHED. Proper DSAEK case selection should include adequate visualization of the endothelium and anterior chamber. Severe stromal haze and edema may prevent necessary surgical observation during the procedure, and a full thickness penetrating keratoplasty may be required.