We present the first report of a patient with atypical hemifacial microsomi
a (HFM) and unilateral aplasia of the floor of the middle cranial fossa, gl
enoid fossa, and portions of her posterior fossa. The patient also develope
d a Chiari I malformation with cervical syrinx over a 3-year interval. This
case report highlights the critical role of imaging in revealing serious,
but clinically occult, structural abnormalities, as well as the evolution i
n the pathogenetic understanding of HFM.