Clinical case: A 32-year-old woman with a history of tumor surgery in the posterior fossa 4 years ago, with a clinical picture of chronic headache and quadriparesis. The magnetic resonance imaging of the cranio-cervical junction showed a cystic lesion at the level of the posterior fossa. A suboccipital craniectomy was performed with removal of the posterior arch of C1 and excision of the tumor. The histopathological study confirmed the diagnosis of enterogenic cyst.

Conclusion: The enterogenic cyst of the nervous system is an uncommon, benign pathology that can recur. The definitive diagnosis will be made by biopsy of the tumor piece.