We studied a case of middle interhemispheric variant of holoprosencephaly by magnetic resonance. A five-year-old child presented a mild language delay and relational disorders. The etiopathogenesis of the disorder remains controversial, the two main hypotheses being teratogenic and genetic. Magnetic resonance disclosed typical alterations (abnormal midline connection of cerebral hemispheres in the posterior frontal and parietal regions, partial agenesis of corpus callosum, absence of septum pellucidum) which allowed differential diagnosis from the other types of holoprosencephaly, namely the more typical alobar, semilobar and lobar variants.
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