The anhydrotic ectodermal dysplasia are malformative syndromes involving the ectodermal components of the organism. They may be associated with very varied ocular manifestations. We described one sporadic case of anhydrotic ectodermal dysplasia in a twenty-four year-old man, involving the following ocular manifestations: severe and bilateral keratopathy corneal hypoesthesia involvement of the lacrymal system horizontal nystagmus and a high rate of antilens antibodies not yet reported a far as we know in the literature.