An enterogenous cyst of the craniospinal region producing medullary compression is reported in a 4.5-year-old boy. The patient presented with stiffness of the neck and headache, but otherwise without neurological deficits. Magnetic resonance imaging (MRI) demonstrated a high-intensity mass extending from the cerebellomedullary cisterna to the second cervical vertebra flattening the medulla and the upper cervical cord. Complete recovery ensued following total excision of the cyst. Histologically, the cyst was lined by a single layer of PAS-positive columnar epithelium. Presentation of this unusual case is discussed together with a review of the literature.