Neurocutaneous syndromes are congenital or hereditary conditions that have many features in common: hereditary transmission, involvement of organs of ectodermal origin (nervous system, eyeball, retina, and skin), slow evolution of lesions in childhood and adolescence, and disposition to fatal malignant transformation. Except for Sturge- Weber syndrome, these major neurocutaneous syndromes are genetically determined, although sporadic cases can occur. This article reviews the clinical features of the more common neurocutaneous syndromes, including tuberous sclerosis complex, neurofibromatosis, Sturge-Weber syndrome, Ehlers-Danlos syndrome, and von Hippel-Lindau disease.