Purpose:The purpose of this study was to determine the clinical characteristics, disease course, therapeutic outcomes, and prognostic factors for pediatric patients with blepharokeratoconjunctivitis (BKC).Methods:A retrospective medical chart review was performed for patients aged 15 years or younger who had been diagnosed with BKC between 2004 and 2020 at 2 tertiary hospitals in Korea. The following data were collected: demographics, medical history, ocular findings, geometric profiling of corneal lesion, medical management, and outcomes.Results:A total of 137 patients (90 female and 47 male) were included. The patients’ mean age was 8.3 ± 3.8 years at disease onset. Both eyes were involved in 57.7% of cases. The most common corneal lesion was corneal neovascularization (77.4%), followed by clinically visible corneal infiltration (51.8%) and stromal scarring (43.1%). Most of the corneal lesions involved a single quadrant, most commonly the inferior quadrant. After treatment, disease remission was achieved in 95% of patients, and visual acuities improved from 0.2 ± 0.3 logarithm of minimal angle of resolution at disease presentation to 0.1 ± 0.3 logarithm of minimal angle of resolution at final follow-up (P = 0.001). Recurrence occurred in 52.6% of patients. Cylinder power was significantly higher in patients with recurrence than in those without. The number of cases of recurrence was positively associated with final cylinder power.Conclusions:Although the treatment induced disease remission in 95% of children with BKC, recurrence occurred in 52.6% of those cases. Because recurrence resulted in significant astigmatism, careful observation for recurrence and prompt management are warranted for preservation of vision in pediatric patients with BKC.