Neurenteric cysts of the spine usually occur at the lower cervical and upper thoracic spine. Neurenteric cyst of the Craniovertebral junction is extremely rare. They are believed to arise from persistent abnormal communication between endodermal and neuroectodermal tissues in the embryonic phase. On T2-weighted MR images, appear as hyperintense cystic lesions without edema is generally observed. An immunohistochemical study is the key to diagnosis and demonstrates the endodermic origin of the lesion. The neurenteric cyst shows epithelial membrane antigen, cytokeratin, and carcinoembryonic antigen. Here we present a rare case of Neurenteric cyst of the Craniovertebral junction diagnosed during third trimester of pregnancy, its management, and outcome with review of literature.
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