Ever since Sorsby described his pseudoinflammatory dystrophy in five families, its characteristics have been unclear. The findings in ten affected members of a seven-generation pedigree are discussed and the literature is reviewed. Patients with this dominantly inherited fundus dystrophy lose central vision between the second and fourth decade of life. Three variations in the fundus appearances were distinguished: in the first and most common, white to yellow fundus spots (which are not drusen) accompany a disciform macular degeneration; in the second, the fundus spots are absent; in the third, the yellow deposits are associated with atrophic macular degeneration. Atrophy of the retina, pigment epithelium, and choroid then slowly progresses toward the periphery. Treatment does not halt the progress of the disease. Although variations in this dystrophy may be examples of genetic heterogeneity, Sorbsy's fundus dystrophy is a distinct clinical disorder.