Inflammatory linear verrucous epidermal nevus (ILVEN) is a unilateral skin disorder which usually manifests in infancy or childhood. Female predominance, frequent leg involvement, pruritus, resistance to treatment, and a psoriasiform histologic pattern are other common characteristics of this disease. Atypical presentations of ILVEN have been described and include late onset in life, widespread involvement and response to treatment. We report a 2-year-old otherwise healthy girl who had bilateral ILVEN and provide a review of this disease.