We report a patient with an inflammatory linear verrucous epidermal naevus (ILVEN) coexisting with an auto-immune lymphocytic thyroiditis. The occurrence of two epithelial inflammatory processes could be linked and raises the question of auto-immune involvement in the inflammatory part of ILVEN. Moreover, expression of a membrane antigen, OKM5, usually assigned to antigen-presenting cells, especially macrophages, has been demonstrated on keratinocytes in some dermatological diseases including ILVEN. These data suggest that keratinocytes in ILVEN could present some antigens and perhaps auto-antigens modified by the hamartomatous process, leading to an (auto-immune?) inflammatory reaction.