[Neurofibroma with contralateral linear hyperpigmentation along Blaschko lines]

We describe a patient with plexiform neurofibroma and contralateral circumscribed hyperpigmentation along the lines of Blaschko. Such findings represent a form of segmental neurofibromatosis. The origin and classification of segmental neurofibromatosis and its relationship to NFI (von Recklinghausen disease) are discussed.