This case report describes carotid-cochlear dehiscence, a rare and possibly fatal condition if missed or ignored on initial work-up of several otologic procedures. Thinning of the bony plate separating the carotid canal from other anatomic structures can occur anywhere along the course of the canal, including the carotid-cochlear bony plate. This condition should be recognized by all otolaryngologists in that it can mimic other otologic pathologies. The aim of this report was to call attention to this condition and its associated mimicking symptoms and inform on its proper management. The idea for this review was formed from the case of a patient who presented with pulsatile tinnitus and was found to have carotid-cochlear dehiscence. Carotid-cochlear dehiscence is a rare anatomic variation of which the neurotologic surgeon should be aware. This condition can mimic common otolaryngologic pathologies that regularly present themselves in clinical settings. We present what we believe to be the fourth reported case of carotid-cochlear dehiscence in the literature. The patient presented having only the complaint of pulsatile tinnitus and was later diagnosed with this rare condition. We advocate a thorough preoperative workup, including high-resolution computed tomography and careful operative planning in a case-specific manner. This is especially important when common pathologies do not become apparent after careful investigation.