• A new, autosomal dominant keratitis is presented. The onset occurs in early childhood with episodes of red, irritated eyes but not recurrent erosions. There are no associated systemic abnormalities. The primary histopathologic features are vascularization and inflammation of the anterior corneal stroma, and replacement of Bowman's layer by fibrovascular tissue. Thus, this disease demonstrates characteristics of both a dystrophy with familial occurrence and early onset, and a degeneration with primary inflammation and vascularization.