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Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare form of epidermal nevus which usually presents in childhood, mostly on the lower half of the body in a blaschkoid distribution. A wide variety of topical, systemic, and surgical modalities have been tried for its treatment, but it is known to be refractory to treatment. We hereby are reporting a rare case of extensive systematized ILVEN which showed moderate response to treatment with acitretin and topical calcipotriol.