Patients with keratoglobus and blue sclera as part of a generalized connective tissue disorder are at a high risk of developing corneal perforations either spontaneously or after mild trauma. Six patients (6 eyes) between the ages of 2 and 16 years of age (mean, 7.5 years) with keratoglobus, blue sclera, hypermobile joints, and consanguineous parents were treated by epikeratoplasty, using commercially prepared 12.5-mm lenticules. Surgery was performed for tectonic support and/or visual improvement and was successful in five of six patients with a follow-up period of 11 to 27 months (mean, 21 months). One lenticule was removed because the epithelium did not heal. Peripheral interface opacities occurred in three patients.