Abstract
Holoprosencephaly (HPE) sequence is a rare spectrum of cerebral and facial malformations resulting from incomplete division of embryonic prosencephalon into diencephalon and telencephalon. De Meyer classified three ranges in order of in- creasing severity: lobar, semilobar and alobar HPE. A subtype called middle inter-hemispheric fissure variant has also been described. Arnold-Chiari III malformation is extremely rare and comprises of occipital encephalocele and associated cerebellar and brain stem abnormalities. This case report presents a male infant with lobar HPE and Arnold-Chiari III malformation without any facial defects and chromosomal abnormalities.
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