The dominant inheritance of a superficial corneal dystrophy was traced through five generations of a family. Eighty percent of those affected had recurrent corneal ulceration during childhood that gradually disappeared in the second and third decades. All of those affected had progressive visual deterioration throughout life. The corneal changes could be seen in seven patients who were photographed on the slitlamp. Previously, Reis-Bucklers dystrophy was often treated by lamellar grafting with poor visual results. Three patients had penetrating corneal grafts that gave excellent visual results.