The authors report a case of a patient with pachyonychia congenita syndrome, a rare genodermatosis inherited as an autosomal dominant trait, who also had otological lesions beyond the other classic signs and symptoms of the syndrome. Many kinds of treatment have already been proposed, but all failed to show satisfactory results. A new, cheap and easy-to-use treatment was developed in this study, using keratoplastics interpolated with humectant lotion for 90 days. The results after three years of follow-up are still thoroughly satisfactory.