Cases of aberrant internal carotid artery in the middle ear have been well documented. However, bilateral aberrant intratympanic carotid artery presentation, as in this case, is quite rare. In addition, in this case, there was unilateral duplication of the cervical and petrous portions of one internal carotid artery such that the duplicated portion rejoined the normal internal carotid artery in its petrous portion. The purpose of this article is to review the literature regarding aberrant internal carotid artery presentation in the middle ear and to discuss the related embryological and anatomical features as well as diagnosis of this lesion, using a unique case as an example.