Radiological features and surgical outcome of a rare neurenteric cyst in Meckel's cave: illustrative case.

backgroundNeurenteric cysts (NECs) are rare, benign congenital lesions of the CNS, typically located in the spinal canal. Intracranial NECs are exceedingly rare, with most cases occurring in the posterior fossa. Only one prior case has been reported in Meckel's cave. OBSERVATIONS: The authors report the case of a 52-year-old woman who presented with acute left-sided headache, nausea, and subsequent horizontal diplopia. MRI revealed a 14 × 9-mm cystic lesion in Meckel's cave with hyperintense signal on both T1- and T2-weighted images, mural calcifications, and no contrast enhancement, suggestive of protein-rich cystic content. Given the presence of an abducens nerve palsy, resection was performed via a left pterional craniotomy. Gross-total resection was achieved. Histopathological examination confirmed an NEC, positive for cytokeratin 7. Postoperatively, the patient experienced transient facial hypoesthesia. Diplopia persisted but was managed with prism glasses. LESSONS: This case highlights the diagnostic and therapeutic challenges of intracranial NECs, particularly in atypical locations such as Meckel's cave. MRI is critical for preoperative assessment, and resection remains the cornerstone of management. Awareness of this rare entity is essential to ensure accurate diagnosis and optimal treatment. https://thejns.org/doi/10.3171/CASE25717.