A case of spinal neurenteric cyst in association with Klippel-Feil deformity, spinal lipoma, and syringomyelia is presented. Neuroradiological evaluation was performed by means of magnetic resonance imaging of the spine and a computed tomography myelograph. The diagnosis of neurenteric cyst was established by histological and immunohistochemical studies. The pathogenesis of syringomyelia associated with an extramedullary mass lesion is discussed.