In this paper we report a 7-month-old boy with lobar holoprosencephaly
in whom midline interhemispheric fusion occured between thickened gyr
i cinguli of both hemispheres at the middle frontal region, This anoma
ly appears to be a variation of a recently recognized type of holopros
encephaly associated with midline interhemispheric fusion and atypical
callosal dysgenesis. in contrast to the previously reported a few cas
es, in our patient the corpus callosum was thinned but intact. III add
ition, the condition was associated with diffuse cortical dysplasia. T
he malformations in this patient is of interest because it contributes
to our understanding of variations of holoprosencephaly.