The clinical presentation and severe complications of atopic keratoconjunctivitis, a potentially blinding disorder, were characterized in a 9-year retrospective study of 45 patients seen at a large referral center. Twenty-six patients had subepithelial fibrosis, 13 with symblepharon and/or fornix foreshortening. Thirty-four patients had severe keratopathy, which included neovascularization in 17 patients. Persistent epithelial defect was another major complication, occurring in 21 eyes and causing severe loss of vision. These patients required interdisciplinary therapeutic strategies; systemic antihistamine therapy and strict environmental controls were the keys to success in most cases.