Dandy-Walker syndrome in monozygotic twins is reported. The twins reported,
presented with delayed development, big head and dysmorphic features. In a
ddition, there were significant cafe-au-lait spots on the trunk and other m
inor features consistent with the diagnosis of neurofibromatosis. To the be
st of our knowledge, Dandy-Walker syndrome in combination with neurofibroma
tosis in monozygotic twins has not been previously reported.