An enterogenous cyst of the craniospinal region producing medullary co
mpression is reported in a 4.5-year-old boy. The patient presented wit
h stiffness of the neck and headache, but otherwise without neurologic
al deficits. Magnetic resonance imaging (MRI) demonstrated a high-inte
nsity mass extending from the cerebellomedullary cisterna to the secon
d cervical vertebra flattening the medulla and the upper cervical cord
. Complete recovery ensued following total excision of the cyst, Histo
logically, the cyst was lined by a single layer of PAS-positive column
ar epithelium. Presentation of this unusual case is discussed together
with a review of the literature.