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Aim: Blepharokeratoconjunctivitis (BKC) is a poorly described entity in children. This study characterises this syndrome in childhood and evaluates epidemiology, clinical grading, and treatment strategies., Methods: 44 children (20 white, 22 Asian, 2 Middle Eastern, median age 5.4 (range 1-14) years) with a diagnosis of BKC were followed for a median of 7 years. Diagnostic criteria included recurrent episodes of chronic red eye, watering, photophobia, blepharitis including recurrent styes or meibomian cysts, and a keratitis. Clinical features were graded as mild, moderate, or severe. The lids and conjunctiva were cultured. The treatment regimen incorporated lid hygiene, topical and/or systemic antibiotics, and topical corticosteroids., Results: The disease was most severe in the Asian and Middle Eastern children (p <0.001), who had a statistically higher risk of subepithelial punctate keratitis (p = 0.008), corneal vascularisation (p <0.001), and marginal corneal ulcerations (p = 0.003), than the white group. 15 children had culture positive lid swabs. Most children had a reduction in symptoms and signs with treatment, and progression of disease after the age of 8 was rare., Conclusions: BKC in children can be defined as "a syndrome usually associated with anterior or posterior lid margin blepharitis, accompanied by episodes of conjunctivitis, and a keratopathy including punctate erosions, punctate keratitis, phlyctenules, marginal keratitis, and ulceration." BKC is common in children in a tertiary referral corneal and external diseases clinic, with the more severe manifestations in the Asian and Middle Eastern populations. Therapy is effective and loss of sight can be prevented in most cases.