AIM: To evaluate the surgical outcome of congenital upper eyelid coloboma repair. · METHODS: All patients underwent complete ophthalmic and general examination before going to surgery, and then examination under anesthesia was performed to assess the site and size of eyelid defect, conjunctival involvement. The status of cornea and ocular motility with forced duction test was also being noted. The surgical procedure was performed according to the size of defect. · RESULTS: Out of 21 cases of congenital upper eyelid coloboma, 18 occurred in isolation with upper eyelid medial defect, 13 were bilateral and 5 were unilateral. Others were associated with Goldenhar syndrome and CHARGE syndrome with bilateral upper lid medial defects. All patients were presented for surgical corrections during age of 2.5-4.0 years except one that presented at 25 years of age. Cosmetically surgical results were acceptable, except one that was already presented with opaque corneal. · CONCLUSION: In this study, overall surgical results were satisfactory except one that was presented late with compromised cornea.