Chiari II malformations and holoprosencephaly have been considered to be br
ain malformations that differ with respect to teratogenic insult, embryolog
ic mechanism, and morphology. We herein describe coexistent Chiari II malfo
rmation and holoprosencephaly that occurred in a viable infant. A review of
the literature regarding Chiari II malformations and holoprosencephaly sug
gests that a disturbance to the mesenchyme in early embryologic life may be
the cause of both malformations.