BACKGROUND: The syndrome of childhood blepharokeratoconjunctivitis (BKC) is frequently underestimated. While prevalent and aggressive among Indo-Pakistani/Middle-Eastern populations, we observe a recalcitrant destructive phenotype in white children/adolescents that persists into early adulthood and may require systemic immunosuppression. METHODS: A cohort of 10 white patients (20 eyes), median age 15.2 (range 6-27) years were identified among 62 patients with BKC attending a tertiary referral centre. Clinical features were graded and lid/conjunctiva swabs were performed, before instituting a hierarchical therapeutic protocol comprising lid hygiene, topical/systemic antibiotics, intensive topical glucocorticoids and systemic immunosuppression. RESULTS: The median duration of symptoms prior to presentation was 4.3 (range 1.2-16.3) years, with 14 eyes (nine patients) demonstrating 360° peripheral corneal vascularisation associated with encroachment/involvement of the visual axis in 10 eyes (six patients). Corneal perforation(s) occurred in three eyes (two patients). Intensive topical glucocorticoids enabled disease control in 10 eyes (seven patients). In six eyes (three patients), persistent active disease necessitated systemic immunosuppression (azathioprine (2), mycophenolate mofetil (1), prednisolone (1)) achieving disease remission within three months with no adverse events reported. CONCLUSIONS: Suboptimal treatment of BKC in white children may permit a progressively destructive sight-threatening phenotype, which may last into adulthood and require immunosuppression. Appropriate aggressive steroid-based and steroid-sparing strategies are vital for disease remission.