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Keratitis–ichthyosis–deafness (KID) syndrome is a rare hereditary disorder caused by the gene GJB2 encoding connexin 26. Patients present the characteristic clinical triad of congenital bilateral sensorineural hearing loss, keratitis, and ichthyosis. Ocular manifestations are corneal neovascularization and severe Meibomian dysfunction associated with hyperkeratotic lid border. Treatments with ocular lubricants, autologous serum, tetracycline, and anti-inflammatory agents have been described. New therapies such as retinoids, gas-permeable contact lenses, or antiangiogenic agents may be indicated. However, sometimes surgical options such as keratoplasty and keratoprosthesis are needed. We report two cases of KID syndrome with different ocular manifestations and management.