To investigate the clinical features and visual outcome of infectious keratitis associated with orthokeratology (Ortho-K) lens in Korean pediatric patients. We retrospectively reviewed medical records of patients diagnosed with Ortho-K lens-related infectious keratitis from June 2005 to April 2020 at a tertiary referral hospital. Patients' demographics, clinical features, microbiological evaluation, and treatment methods were assessed, and factors related to final visual outcomes were analyzed. The study included 26 eyes from 26 patients (19 female and 7 male patients; mean age, 11.9 years), with an average Ortho-K lens wear duration of 33.7 ± 21.2 months. The highest number of cases occurred in summer (11 of 26 cases, 42.3%). Central or paracentral corneal lesions were observed in 25 cases (96.2%), with a mean corneal epithelial defect size of 5.13 mm2. Pseudomonas aeruginosa was the most commonly isolated organism (n = 5), followed by Serratia marcescens (n = 4). All patients responded to medical treatment without needing surgical intervention. 72% of cases achieved favorable visual outcomes (Snellen best-corrected visual acuity [BCVA] >6 / 12), while 8% experienced severe visual impairment (Snellen BCVA ≤6 / 60) due to residual central corneal opacities. Multivariable analysis showed that non-summer seasons (p = 0.043), duration from symptom onset to presentation (p = 0.040), and corneal epithelial defect size (p = 0.002) were significantly associated with final logarithm of the minimum angle of resolution BCVA. Failed autorefraction at presentation due to an Ortho-K-related infectious keratitis lesion was a significant predictor of poor final visual outcome (Snellen BCVA ≤6 / 12; odds ratio, 38.995; p = 0.030). Ortho-K lens-related infectious keratitis can lead to permanent corneal opacities and potentially devastating visual outcomes in children. Delayed time to presentation, large corneal lesions, failure of autorefraction, and non-summer seasons were associated with poorer outcomes. Proper education and early detection would be key to safe use of orthokeratology lenses in pediatric patients.