Epidermal nevus syndrome is defined as a combination of nevoid skin alterations, epileptic seizures, and psychomotoric retardation. Involvement of other organs, especially the eyes, may also occur, but is not obligatory. The present report is concerned with a case that combined regional odontodysplasia with nevoid skin alterations in the overlying skin area of the face. This observation suggests that odontodysplasia may be the result of aberrations in the development and migration of cells of the neural crest occurring early in embryonic life.