Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare and chronic skin disorder, which may trouble the patient considerably. The condition is generally believed to be resistant to therapy, although some authors have reported success with several treatments, including dithranol and etretinate. The present case, a classical presentation of ILVEN, again illustrates the refractoriness to various treatments, including an experimental treatment with topical 13-cis-retinoic acid. A review of the literature on therapeutic possibilities of ILVEN is presented. Based on our own observations and literature data, it is attractive to hypothesize that a positive result with treatments such as dithranol and retinoids should be interpreted as an antipsoriatic effect in ILVEN with superimposed psoriasis.