Background: We report a 61-year-old who presented with a right-sided abducens nerve palsy secondary to a middle cranial fossa adenoid cystic carcinoma (ACC) extending into the cavernous sinus. Purpose: This case represents a unique presentation of intracranial ACC with a large middle cranial fossa mass and only a small extracranial component. Methods: Review of the literature was undertaken to identify cases of intracranial ACC and their range of presentations. Results: Our results show that this is the first reported case of an ACC presenting mostly as an intracranial mass with an isolated cranial nerve lesion. Conclusion: Our case highlights the importance of a broad differential diagnosis, particularly in circumstances where there are atypical features of lesions on radiographic imaging.