Aberrant internal carotid artery is rarely seen in the middle ear cavity. Aberrant internal carotid artery can mimic dehiscence of the jugular bulb, glomus tumours, haemangiomas, and cholesterol granulomas on computed tomography. Because the symptoms and signs are usually nonspecific or absent, clinical diagnosis of aberrant internal carotid artery is very difficult. The most common symptoms are pulsatile tinnitus, hearing loss, and retrotympanic mass. In this case, we report an aberrant internal carotid artery in the middle ear presenting with pulsatile tinnitus and hearing loss.