An enterogenous cyst of the spinal canal has the interior wall composed of tissue that is similar to the epithelium of the digestive or respiratory organ system, and is considered to be produced by a developmental abnormality in the early embryonal period. This is a rare disease, and this time we have experienced a case of an enterogenous cyst, occurring in the cervical canal, with a favorable course with improvement of symptoms after an operation.The case is a 13-year-old female complaining of numbness and weakness of the right hand, weakness of the right lower limb, and disturbance of gait. Present illness revealed stretch gymnastics from the beginning of December. She developed upper back pain, then pain in the ulnar side of the right hand, weakness in the right hand and lower limb, inability to stand up, and disturbance of urination. She was admitted as an emergency on 31 Dec. From the findings of myelography, CTM and MRI, an intradural extramedullary cystic tumor was suspected. Total excision was attempted from the posterior approach, but the wall ruptured and the tumor reduced. Only the wall was excised, the pathological examination revealed irregular papillary tissue composed of a single cubical-cylindrical epithelium, or partly stratified arrangement with cilia, suggesting an enterogenous cyst. At present she is back to school.