Abstract Background Ameloblastoma (AMBL) is an odontogenic tumor, considered to be benign, but aggressive, whose principal risk is a recurrence. The growth can be enormous, and it can extend into the intracranial compartment with serious consequences. Purpose The intracranial involvement of AMBL is rare, and it may require an extensive surgery. Although it is a rare condition for the neurosurgeon to treat, knowing this condition can lead to a significant increase in survival for these patients. Methods A case of a 56-year-old woman presented with a history of recurrent left maxilla AMBL with intracranial extension and dural involvement of the anterior and medial cranial fossa is reported, followed by a systematic review of the literature with the aim to identify the best surgical treatment. Results A total of 32 cases were included in the qualitative analysis. Management is varied and often not described, resulting in an almost complete lack of information and indications for treatment. Radical surgery tends to yield the best outcomes, and it is recommended to have adequate surgical margins when possible. Conclusions Intracranial involvement from AMBL compartment is an uncommon manifestation of this rare pathology, but which deserves to be treated in a multidisciplinary way in order to ensure maximum surgical radicality. Recurrence reflects failure of the primary surgical resection. If recurrence is the major consideration, surgeons are encouraged to select radical surgery. Whenever a follicular-type maxillary AMBL is diagnosed, it is advisable to check for intracranial spreading and distant metastases during follow-up.