The case of an 11-year-old Japanese girl with an intradural and extramedullary enterogenous cyst is presented. A mass giving a low-intensity signal in comparison with the spinal cord was demonstrated on magnetic resonance imaging. Histologically, the diagnosis was confirmed on specimens stained with periodic acid-Schiff, alcian blue, mucicarmine, and immunohistochemical staining of carcinoembryonic antigen, and by electron microscopy.