Progression of the maculopathy in North Carolina macular dystrophy (NCMD) was not well documented. Thus, the author recently examined 22 affected members of the original kindred. Evidence of progression of the macular disease was sought through comparison of the recent fundus findings with old fundus photographs and from subjective complaints of worsening visual acuity. Only 1 of the 22 affected subjects had evidence of such change. Additionally, two new findings of NCMD were observed: (1) severe macular lesions which were staphylomatous or excavated in appearance, not flat, and atrophic as previously described; and (2) peripheral retinal drusen variably present in affected subjects, in contrast to the "normal peripheral retina" originally described. These new findings, along with the generally stable course of the disease would seem to alter our understanding of the relationship of NCMD to other dominant macular dystrophies.