Congenital hereditary endothelial dystrophy (CHED) has historically been managed with penetrating keratoplasty (PK), with moderate success, and, more recently, with Descemet's stripping endothelial keratoplasty (DSEK). The possibility of repeated graft failures with CHED, however, makes alternative surgical procedures desirable. To our knowledge, this is the first reported use of a keratoprosthesis for management of CHED in a patient with multiple graft failures. The patient has been successfully followed for 5 years, has 20/30 vision, and no glaucoma.