The epidermal nevus syndrome is a disease complex of epidermal nevi and dev
elopmental 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 dio
xide laser utilizing two different techniques. The larger verrucous lesions
were debulked initially, and the resulting defects and thinner lesions wer
e treated using the Silk Touch modality. The lesions were dermaplaned seque
ntially until they were ablated completely. All wounds healed without compl
ication, and in a 2-year follow-up the patient has experienced no recurrenc
e in the treated areas. Epidermal nevi can be treated safely, effectively,
and without recurrence with carbon dioxide laser ablation.