The neuroncristopathies, originally defined in 1974 as a category of d
iseases arising in neural crest development are reviewed as to their c
urrent status. Accompanying the great advances in neural crest ontogen
y, there has been an increase in the number and variety of neurocristo
pathies, particularly in the definition of craniofacial syndromes deri
ved from the cranial crest mesectoderm. Molecular biology and genetics
have added new dimensions in defining interrelationships between a nu
mber of as a pathogenetic concept should continue to be useful as a st
ructural framework for future investigations.