The epidermal nevus syndrome is a disease complex of epidermal nevi and developmental abnormalities of different organ systems. The authors present a case of congenital systematized epidermal nevus syndrome in a patient with skin lesions covering approximately 80% of the total body surface area. The patient underwent staged treatment of the epidermal nevi with a carbon dioxide laser utilizing two different techniques. The larger verrucous lesions were debulked initially, and the resulting defects and thinner lesions were treated using the Silk Touch modality. The lesions were dermaplaned sequentially until they were ablated completely. All wounds healed without complication, and in a 2-year follow-up the patient has experienced no recurrence in the treated areas. Epidermal nevi can be treated safely, effectively, and without recurrence with carbon dioxide laser ablation.