Neurenteric cyst is a rare congenital anomaly, which is included in the split notochord syndrome. The cyst arises from abnormal persistence of the neurenteric canal at about three weeks of gestation. The most common locations are extramedullary intradural. They can extend anywhere along the neuraxis. The intermittent, polymorphic and misleading clinical symptoms may result in a delay in diagnosis. Magnetic resonance imaging is the gold standard in the diagnosis of this anomaly. Surgical treatment allows total recovery in most cases.