Neurilemmomas of the 12th cranial nerve are rare tumors. We present the first reported case of one occurring in the middle ear. This report details the diagnosis and treatment of a large 12th nerve neurilemmoma in a patient whose initial symptoms included vertigo, otalgia, and left-sided tongue fasciculations with atrophy. Selective carotid angiography and computed tomography scanning defined the extent of the lesion. It involved the skull base and parapharyngeal space with extension into the middle ear and mastoid to the level of the middle fossa and posterior fossa dura. Treatment consisted of total excision via a Blair incision with a retroauricular limb, which is applicable to many skull base and parapharyngeal tumors.