This study was to report the outcomes of Descemet stripping automated endothelial keratoplasty (DSAEK) in managing corneal endothelial disorders in children less than 14 years of age. Medical records of 180 DSAEK performed, between 2008 and 2020, on 167 eyes of 111 children who had a visually significant endothelial dysfunction were retrospectively reviewed for the primary etiology of corneal decompensation, time of the onset and duration of cloudiness, preoperative visual acuity, the technique of endothelial keratoplasty, surgical modifications, and reintervention. Median age at surgical intervention was 7.9 years (interquartile range, 5.2-11.2 years). At a median postinterventional follow-up of 2.5 years (interquartile range, 0.9-3.4 years), 86.2% (144 of 167 eyes, 95% confidence interval 79.9%-90.1%) maintained a clear graft. Median best-corrected visual acuity (logarithm of the minimum angle of resolution) improved from 1.45 ± 0.70 preoperatively to 0.90 ± 0.06 at the last follow-up visit (P < 0.0001). The cumulative overall long-term graft survival was 92.7%, 86.5%, and 77.7% at 1, 3, and 7 years, respectively. Cox proportional hazards regression analysis showed that the indication for DSAEK (P = 0.007; hazards ratio: 2.17 ± 0.62), age at surgery (P = 0.02; hazards ratio: 0.87 ± 0.05), and any subsequent intervention after DSAEK (P = 0.003; hazards ratio: 0.11 ± 0.08) were significant risk factors for DSAEK failure. The endothelial cell loss was 40.1% at 6 months, 45.4% at 1 year, 55.2% at 5 years, and 61.9% at 7 years. DSAEK is a safe and effective surgical strategy in managing endothelial disorders among the pediatric age group.