Neurofibromatosis (NF) has perhaps been the most notorious of the neurocuta
neous disorders. Both Quasimoto of Victor Hugo's The Hunchback Of Notre Dam
e and John Merrick, known as the Elephant Man (who subsequently has been mo
re properly classified as having Proteus syndrome, a quite different disord
er), are 2 infamous examples that have shaped many popular misconceptions a
bout this disease. Neurofibromatosis is now understood as a hamartomatous d
isorder on the basis of molecular genetic studies. Studies on NF have been
pivotal to understanding the functions of oncogenes in rumorigenesis.