Objective: Chronic otitis media is a potentially serious disease because of its complications, most of which are common in conjunction with cholesteatomas. There is variance in the terminology used by neurosurgeons, otorhinolaryngologists and neuropathologists. Synonyms for cholesteatoma found in the literature include epidermoid tumor, epidermoid cysts and epithelial inclusion cyst. Intracranial extension of an acquired cholesteatoma is a rarely documented occurrence. Patients/Material and Methods: A 47-year-old woman who had undergone a right tympanomastoidectomy 20 years previously, presented with a long history of mild headaches that had become progressively more severe over the last 3 years. Clinical and radiological evaluation of the patient raised the suspicion of an intracranial cholesteatoma. Results: A right temporal craniotomy was performed. The mass was completely excised and histopathological study revealed the tumor to be a cholesteatoma. The patient's post-operative recovery was uneventful. Conclusions: Cholesteatomas possess the capacity for eroding bone and can have an insidious onset, but once established, grows relentlessly and destroys neighboring structures. Since a gradual intracranial involvement does not usually cause acute symptoms of increased intracranial pressure, the correct diagnosis may be difficult. Detailed clinical and radiographic studies in particular are diagnostically helpful. Because of the high incidence of delayed recurrence, life-long follow-up is required. The inconsistency in the histopathological classification of intracranial cholesteatomas should be clarified.