To analyze the clinical results and potential influential factors of modified large-diameter lamellar keratoplasty in patients with total limbal stem cell deficiency. Thirty-six eyes of 35 patients were included in this study. Best-corrected visual acuity, ocular surface stability, central corneal graft clarity, and postoperative complications were recorded. Related influential factors were evaluated using Cox regression analysis. At the final follow-up (mean, 48.1 ± 43.2 months), 21 eyes (58.3%) had an improvement in best-corrected visual acuity of at least 1 line. Twenty-seven eyes (75.0%) had a stable ocular surface. The survival of ocular surface stability was 74.4% ± 7.4%, 58.9% ± 8.5%, 51.1% ± 9.0%, and 39.3% ± 9.2% at 1, 2, 5, and 7 years after surgery, respectively. Twenty-one eyes (58.3%) maintained a clear graft. The survival of central graft clarity was 74.4% ± 7.4%, 59.5% ± 8.4%, 55.2% ± 8.8%, and 46.7% ± 9.3% at 1, 2, 5, and 7 years after surgery, respectively. Thirteen eyes (36.1%) developed persistent epithelial defects, and immune rejection occurred in 9 eyes (25.0%). The Cox regression analysis revealed that previous eyelid reconstruction [relative risk (RR) = 0.035] and combination with subconjunctival implantation of a cyclosporine A drug delivery system (RR = 0.170) were protective factors. Preoperative persistent epithelial defects (RR = 3.443) and decreased Schirmer test (RR = 6.770) were risk factors associated with ocular surface stability. The modified large-diameter lamellar keratoplasty seems to be effective in reconstructing an ocular surface with long-term stability. Improvement in tear production, reconstruction of eyelid abnormalities, and combination with subconjunctival implantation of an immunosuppressive drug delivery system can help to maintain a stable ocular surface.