Objective: To report a patient with large neurenteric cyst located in the anterior cranial fossa presented with uncommon
presenting symptoms.
Case Report: A 47-year-old healthy Thai male presented with gradually decreasing vision in the past six months and esotropia
for one year. Other neurological examinations and pituitary hormone tests were unremarkable.
Results: Orbital magnetic resonance imaging (MRI) depicted a large lobulated cystic mass with the size of 7.1x8.0x7.5 cm,
abutted on the sellar, suprasellar, anterior cranial fossa regions, and bilateral small size optic nerves. Aspiration and incisional biopsy of the cyst were performed. Tissue pathology was compatible with neurenteric cyst.
Conclusion: Neurenteric cyst is rarely located in the anterior cranial fossa. Although this patient had a very large lesion
located in anterior cranial fossa and sellar area, he had only ophthalmic symptoms without headache, nausea, or any
neurologic deficits. It is important not to overlook this rare cyst with an unusual presentation.

Keywords: Neurenteric cyst, Anterior cranial fossa, Endodermal cyst, Intracranial, Visual loss