Introduction This paper reports 97 eyes (49 patients), all showing a group of intraocular pigment changes due to an idiopathic atrophy of the retinal layers of the iris and probably the ciliary body, which we believe comprise a single clinical entity. The condition is often associated with glaucoma. Most of the pigment changes are well known, but usually they have been described with little or no attempt to correlate them into a disease complex. They consist of (1) punctate pigment deposition on the corneal endothelium, usually in the form of a Krukenberg spindle; (2) speckling of the anterior surface of the iris with pigment; (3) pigmentation of the trabecular area of the anterior chamber; (4) pigment on the posterior surface of the lens near the equator, occasionally in the form of a ring, and (5) atrophy of the epithelial layers of the iris. Glaucoma that follows a chronic simple (open-angle)