A 13-year-old boy with an intradural extramedullary enterogenous cyst is presented. He presented with progressive weakness of lower limbs. The myelography and computed tomography scan of spinal cord demonstrated an intradural, extramedullary lesion at the spinal level of T 7-9. A cystic lesion was discovered during operation. The cyst caused the spinal cord compression and was related to neurological deficit. Histologically, it was confirmed by Periodic Acid-Schiff and Alcian Blue stain to contain mucin. He recovered very well after surgical removal of the cyst.