<h3>Introduction</h3> This rare hereditary retinal dystrophy has not been reported previously in this country, although several reports have appeared in the British and European literature.<sup>1-7</sup>The name "central senile areolar choroidal atrophy" was appended to the disorder in 1884 by Nettleship<sup>1</sup>who first described this entity; and other authors,<sup>4,5,8</sup>on the basis of the clinical picture, have used the term "central areolar choroidal sclerosis." However, the genetic nature of the disorder and the absence of a circumscribed sclerosis of the choroidal vessels make the term "central areolar choroidal dystrophy" preferable. <h3>Report of Cases</h3><h3>Case 1 (Fig 1 and 2).</h3> —This 57-year-old white female, the mother of patients 2 and 3, has had a bilateral decrease in visual acuity since age 14. This was of very rapid onset, and there was no progression of the symptoms after age 20. She prefers a darkened environment for good visual acuity.