Purpose: To report the utility of preoperative anterior segment optical coherence tomography (ASOCT) in decision-making for stripping of the Descemet membrane (DM) during Descemet membrane endothelial keratoplasty (DMEK) in eyes with congenital hereditary endothelial dystrophy (CHED). Methods: ASOCT was performed in eyes with CHED undergoing DMEK. Host DM was retained if the DM appearance was normal on ASOCT. Outcomes of DMEK with or without DM stripping (non DM stripping [DMEK]) were analyzed and compared regarding graft adhesion, graft clarity, visual acuity, and complications. Results: Twelve eyes of 8 patients were included in the series. Of these, 6 eyes (mean age, 6 +/- 2.6 years, range 3-8 years) underwent nDMEK and 6 eyes (mean age, 23.6 +/- 10.2 years, range 13-39 years) underwent standard DMEK. There were no intraoperative complications in either group. During the early postoperative period, one eye in the nDMEK group had donor detachment that was successfully managed by repeat air injection. The mean preoperative and postoperative corrected visual acuities were 1.15 and 0.37 logarithm of the minimum angle of resolution (LogMAR) in the nDMEK group, and 0.95 and 0.25 LogMAR in the DMEK group (P 0.39 and 0.06). Average endothelial cell counts were 1826 +/- 318 cell/mm(2) in the nDMEK group (32.3% loss), and 1708 +/- 271 cells/mm(2) in the DMEK group (33.6% loss) at the last follow-up. Conclusions: Preoperative anterior segment OCT is useful in decision-making regarding stripping of host DM during DMEK in eyes with CHED. The outcomes of nDMEK were similar to DMEK in this small series.