The patient is a 64-year-old woman who developed a sensation of right ear fullness and hearing loss in early November 2010. Physical examination revealed a painless reddish granular lesion filling in the right external auditory canal. Her right ear was deaf, and no facial palsy was noted. Computed tomography, magnetic resonance imaging and positron emission tomography revealed a middle ear mass extending to the external auditory canal with intracranial invasion causing temporal lobe retraction and inferiorly extending just anterior to the jugular bulb as well. A combination of transmastoid and middle cranial fossa approach along with anterior rerouting of the facial nerve was employed for a near-total removal of the tumor. Based upon the operative findings, it was deemed that the tumor could have arisen from the Jacobson's nerve.