SUMMARY A 2-year-old child was referred because of a prominent blind left eye with an opaque cornea. Although extraocular extension of a retinoblastoma was an initial diagnostic possibility, computed tomography (CT) showed only a large globe with a corneal staphyloma. Because of the cosmetically unacceptable appearance the involved eye was enucleated. The clinical, CT, and histopathological features of congenital corneal staphyloma are discussed, as well as the possible pathogenic mechanisms.
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