The inflammatory linear verrucose epidermal nevus is a recently individualized special type of linear nevus. The particular features of this nevus are represented clinically by the pruritus and the erythematous, scaly, at times psoriasiform and frequently lichenifled aspect of the papulokeratotic lesions. Histologically, this disease is characterized by a psoriasiform picture or that of chronic eczema with, in some instances, the alternation of areas of agranulosis and hypergranulosis accompanied, respectively, by overlying parakeratosis and orthokeratosis. These areas are very sharply separated. The authors report a case and discuss the clinical and histologic criteria which justify the autonomy of this nevus, as well as the differential diagnosis, the etiopathogenesis, its relation to the ‘epidermal nevus syndrome’ of Solomon et al. and its treatment.