This is a typical case of Hallermann-Streiff-Francois Syndrome in which the glaucomatous complication may be caused by developmental malformations in the anterior segment of the eye. Bilateral trabeculectomy succeeded in controlling the tension after a follow-up of two years. Glaucomatous complication seems to be frequent and as important as the other described cardinal symptoms. The histologic examination of the skin confirmed the atrophic changes that were observed clinically.