Neurenteric cysts (NEC) are rare congenital abnormalities of the spine that usually happen when there is an abnormal connection between the primitive endoderm and ectoderm. This happens when the proper separation of the endoderm and notochord is prevented by the persistence of the ordinarily transitory neurenteric canal, and this connection leads to the presence of mucus-secreting epithelium reminiscent of the gastrointestinal tract. The clinical presentation of NEC can be subtle and is dependent on the cyst’s location on the spine. Diagnostic imaging and histopathology are performed in addition to a detailed history taking and physical examination for the proper diagnosis of this condition. In this chapter, we gathered and described NEC from pathogenesis to clinical presentation, diagnosis to clinical management, and prognosis and clinical outcome.