Background: Spinal intramedullary ependymal cysts are extremely rare. Only seven pathologically proven cases have been reported in the literature.
Method: We present an 18-month-old female with thoracic spinal intramedullary ependymal cyst that was diagnosed pathologically.
Results: Histological diagnosis was made by light microscopy after immunostaining. After partially removing the cyst wall and establishing communication between the cyst and the subarachnoid space, the patient improved neurologically.
Conclusions: For spinal intramedullary ependymal cyst we recommend diagnosis by MR imaging without myelography, then enucleation of the cyst, if possible. Otherwise, we remove the cyst wall as much as possible and create adequate communication between the cyst and the subarachnoid space.