To present a rare case of glial heterotopia in the middle ear occurring in association with cholesteatoma.

A 56-year-old female presented with right-sided hearing loss and otorrhea. ENT examination and CT imaging revealed middle ear cholesteatoma. Right canal wall down mastoidectomy with type III tympanoplasty revealed a well-demarcated, soft, pinkish mass in the right middle ear without tegmen tympani dehiscence. Histopathological analysis confirmed glial heterotopia.

Complete cholesteatoma resection, resolution of otorrhea, absence of neurological complications, and right-sided hearing improvement.

The patient demonstrated an uneventful postoperative recovery. At the five-month follow-up, tympanoplasty outcomes remained stable. Repeat imaging confirmed absence of skull base defects, with clinical resolution of otorrhea. No postoperative neurological complications (including cerebrospinal fluid leakage) were observed.

Middle ear glial heterotopia is a rare entity that may be discovered incidentally or in association with infection/cholesteatoma. Definitive management requires surgical excision of the heterotopic tissue and concurrent repair of skull base bone defects when identified. Preoperative imaging evaluation with CT or MRI can be valuable to delineate anatomical relationships and exclude intracranial connections.