BACKGROUND: Congenital melanocytic nevi of the eyelids and periorbital region are unusual. Although their malignant potential can be debated, they present a significant aesthetic concern and also disturb lid function. In this article, the authors present an expanded approach to evaluation and treatment of these patients. METHODS: Forty-four consecutive patients, aged 6 months to 18 years, were treated from 1980 to 2008. All patients had congenital nevi involving one or both eyelids, with or without extension into the surrounding periorbital area and face. Follow-up ranged from 6 months to 20 years. RESULTS: All patients were treated successfully with excision and reconstruction of their congenital eyelid and/or periorbital nevi. The involved ciliary border was preserved in all but one case, where the exophytic lesion presented function concerns. Complications included asymptomatic lateral ectropion in three patients. Asymmetry of the palpebral apertures, before treatment, was present in at least half of the patients with extensive facial nevi, and the abnormalities causing these differences may impact efforts to obtain final lid symmetry. A single patient died as a result of extensive metastatic melanoma from an extracutaneous site. CONCLUSIONS: Early evaluation and treatment of these nevi may help in preventing the aesthetic, functional, and health-related issues for the patients. Although the current group of infants and young children will not reach full facial growth for more than another decade and a half, and therefore await critical assessment of their long-term outcomes, the authors hope that the experience gained to date will assist surgeons in managing these complex reconstructions.