A condition causing glaucomatous optic atrophy and visual field loss at normal or borderline intraocular pressure affected eight members of a family of consecutive generations. The disease was detectable in early adulthood and progressed slowly throughout life. The pattern of inheritance is autosomal dominant. One affected individual died of a myocardial infarction, and his eyes were obtained post mortem. Light and electron microscopic examination demonstrated glaucomatous optic atrophy with loss of ganglion cells. The trabecular meshwork, choroidal and optic nerve vasculature, retinal pigment epithelium, and photoreceptors were normal in appearance. We believe this family has an autosomal dominant genetic condition that is a distinct type of low-tension glaucoma.