Purpose: Epithelial cysts of the central nervous system, according to the type of cell origin; neuroepithelial or endodermal type. Endodermal cysts are rare forms of benign tumors. In this presentation, we present a 35-year-old male patient with typical histopathologic features and due to the presence of this rare lesion. We aimed to present this rare case and emphasize the importance of cystic epithelial cell differences.

Methods: Neurenteric cysts, cyst lining epithelium of cell types were analyzed using immunohistochemical diagnostic methods.

Results: The importance of the cell type in the endodermal cyst is also emphasized and the frequency of recurrence in cysts that have goblet-columnar epithelial component. This epithelium is increasing the risk of recurrences. However, the other cause of recurrence is also incomplete excision. Therefore, the complete excision of ECs is important, not only for avoiding recurrence but also for differentiating cell types for ECs and predicting recurrence risk.

Conclusions: The precise pathologic diagnosis of cell type determines the risk of recurrence. Neurosurgeons should exert their best efforts to achieve complete excision of the cysts.

Keywords: Endodermal cysts, Immunohistochemical, Pathogenesis