Objective and importance: We report a case of a neurenteric cyst of the cerebellopontine angle and review the five previously reported cases. The pathology and classification of these cysts are discussed.
Clinical presentation: The patient presented with a 1-month history of nausea, vomiting, vertigo, and sudden hearing loss.
Intervention: The cyst was decompressed by a retrosigmoid approach. After recurrence of symptoms at 2 months, further decompression was required.
Conclusion: The patient achieved a good outcome after the second operation, with cessation of her vomiting and vertigo, although she had residual hearing loss. Four of the five previously reported patients experienced satisfactory outcomes after surgery. The definitive diagnosis of these rare lesions is made using immunocytochemical techniques.