To describe the outcomes of Descemet membrane endothelial keratoplasty (DMEK) performed in amblyopic aged (younger than 8 years) children. This is a single-center retrospective study, including 11 eyes (7 congenital hereditary endothelial dystrophy and 4 congenital glaucoma) of 6 children in amblyopic age undergoing DMEK by a single surgeon (N.C.P.) at Sorocaba Eye Hospital from December 2015 to November 2017. Best spectacle-corrected visual acuity, biomicroscopy, pachymetry, endothelial cell density, and complications were evaluated. No intraoperative complications occurred. Graft detachment occurred in 1 eye (9.1%) and was successfully managed with rebubbling. No primary graft failure or pupillary block was observed. All pachymetric measurements improved, and the corneal edema clinically resolved in all eyes within 2 weeks after the procedure. At the last follow-up (mean 30 months), best spectacle-corrected visual acuity was ≥20/40 in 7 (77.8%) of 9 eyes from patients cooperative enough to assess vision. All children began visual stimulation therapy and amblyopic treatment within 1 month of surgery, and all grafts remained clear until the last follow-up. The mean preoperative donor endothelial cell density was 2588 ± 236 cells/mm, which decreased to 1726 ± 292 cells/mm 2 years after surgery, yielding a 33% reduction (P < 0.001). No immunologic graft reaction, secondary graft failure, or cataracts were observed during the follow-up period. In this series, DMEK was performed to successfully treat endothelial dysfunction in children. However, the procedure is more challenging, and more studies with more patients and longer follow-up are needed to confirm the superiority of DMEK in treating endothelial dysfunction in children.