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Neurenteric cysts are rare congenital lesions of the spine that are lined with endodermal epithelium. Their most common location is the cervico-dorsal region, and the mass usually lies ventral to the spinal cord. However the conus medullaris area location is an uncommon location. Neurenteric cysts are best treated by decompression and as near total excision of cyst membrane as possible. We report a case of a 7 year-old-girl with a neurenteric cyst in the conus medullaris. The patient had a history of meningitis and a gait disturbance. Magnetic resonance imaging (MRI) showed an intramedullary mass lesion in the conus medullaris with syringomyelia. There was no associated bone or soft-tissue anomaly. The mass was subtotally removed through a posterior approach. However 4 months later, meningeal irritation signs developed and MRI showed recurrence of the cyst. At the second operation, the cystic membrane was totally removed and the patient's neurological symptoms improved postoperatively. We reports a case of recurred neurenteric cyst occurred in unusual location with the review of literature. KEY WORDS : Intramedullary neurenteric cyst·Conus medullaris·Recurrent meningitis.