Spinal neurenteric (NE) cyst is an uncommon congenital cyst and frequently found in the cervical region. The clinical symptoms associated with this entity depend on the site of the lesion and are not typical for all such cysts. A definitive diagnosis can only be made by biopsy and histological examination. MRI can confirm these cystic masses and is the method of choice for their imaging investigation. They are often connected by a fibrous tract, fistula or cleft to structures derived from the primitive gut in the thoracic or abdominal cavities and are commonly associated with anterior spina bifida or other vertebral anomalies. We report a case of craniocervical NE cyst without associated abnormalities and discuss the implications for clinical diagnosis and management by a thorough review of the literature.

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