Purpose: Neuroepithelial cyst is considered an unusual differential diagnosis for cysts in the posterior fossa. Here, we present a paediatric case with such a pathology and review the pertinent literature. Methods: A 12-year old girl with headache, vertigo and disturbed gait was diagnosed with a cystic lesion in the fourth ventricle after brain MRI study. She was operated with the pre-operative diagnosis of arachnoid cyst. Results: A transparent, colourless cyst was observed intra-operatively. As frozen sections were consistent with endodermal cyst, total removal of the cyst was attempted. Definite histopathological studies and immunohistochemistry stains were in favour of neuroepithelial cyst. No regrowth of the cyst or recurrence of the symptoms was observed in her 2-year follow-up. Conclusions: As neuroepithelial cyst is rarely encountered in the posterior fossa, the clinical, radiological and pathological characteristics of our case along with similar cases in the literature were reviewed and discussed.
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