AIM: To summarize preoperative evaluation and outcome of corneal transplantation for limbal dermoids for ten years. METHODS: Eighty-five patients diagnosed with limbal dermoids and treated with corneal transplantation were analyzed retrospectively. All patients were further divided into two groups according to absence or presence of neovascularization surrounding the dermoids in the corneal stroma. Eighty-two eyes were treated with tumor excision combined with partial lamellar sclerokeratoplasty, and the other three eyes were performed by penetrating keratoplasty. The size and location of the tumor, the associated ocular and systemic anomalies, the depth of the corneal penetration of tumor tissues, the preoperative and postoperative best-corrected visual acuity (BCVA), graft survival and cosmetic outcome, and surgical complications were recorded respectively. RESULTS: The average age at surgery was 5.3y (range, 3mo-36y). The mean size of dermoids was 6.1±1.6 mm. The 43.5% of eyes (37/85) were present with hair at the surface of the dermoid and 72.9% of dermoids were located inferotemporal of the eye. Amplyopia was present in 34.1% of patients (29/85) and 9.4% of patients (8/85) had lipodermoids. Eighteen patients suffered from Goldenhar’s syndrome with an accessory ear. The 75% of patients in group 1 had involvement of the corneal deep stroma down to Descemet’s membrane without involving it, but 71.4% of patients had Descemet’s membrane involvement in group 2. Preoperative BCVA ranged from counting fingers to 20/20. Postoperatively 81.1% had a BCVA of 20/800 or better. There was no significant difference between the post-surgical BCVA of the two groups (t=1.584, P>0.05). The grafts of 70.5% patients were present as 1+ opacity, 21.1% as 2+ opacity, 8.2% as 3+ opacity and none as 4+ opacity. Surgical complications included graft rejection, microperforation, prolonged reepithelialization, steroid glaucoma, interface neovascularization, and interface hemorrhage. CONCLUSION: The dermoids with neovascularization surrounding them in the corneal stroma invaded deeper tissues in the cornea than those with no neovascularization surrounding them in the corneal stroma. Therefore, surgeons should take care to avoid corneal perforation during the corneal transplantation operation. The majority of patients markedly improved their cosmetic appearance after surgery.