A patient with anhidrotic ectodermal dysplasia and severe dry eyes was admitted with spontaneous corneal perforation in his right eye. The corneal perforation was sealed with cyanoacrylate glue and bandage contact lens placement. Three weeks later, the patient underwent penetrating keratoplasty. On follow-up examination, he had corneal graft abscess that was treated successfully with topical antibiotics and corneal erosion that was treated with a bandage contact lens. Corneal topography performed as a routine examination showed inferior corneal steepening typical of keratoconus in the left eye. This accidental discovery of keratoconus in the patient's left eye may suggest a possible association between anhidrotic ectodermal dysplasia and keratoconus.