Intramedullary neurenteric cysts (NEC) without associated malformations are extremely rare and, to our knowledge, have never been reported in association with calcification. We report a unique imaging presentation as a partially calcified mass of an isolated intramedullary neuroenteric cyst of the lower thoracic spinal cord with pathologic correlation. The literature for isolated forms of intramedullary NEC since the advent of magnetic resonance imaging is also reviewed.