An aberrant internal carotid artery (AICA) is a rare vascular anomaly. Misdiagnosis may lead to massive hemorhage or other life threatening complications during even a minor surgical procedure of the tympanic membrane or middle ear. Knowledge of this rare entity is essential to any surgeon who undertakes myringotomy and middle ear surgery. Only 14 cases of bilateral AICA were reported in literature. We present a very rare case with bilateral AICA presenting with pulsatile middle ear discharge from his left ear and left sided conductive hearing loss. Tympanic membrane perforation and middle ear discharge were evaluated inaccurately as chronic otitis media on otoscopic appearance. The definitive diagnosis made after CT (computed tomography) and MRA (MR angiography). Otolaryngologists should keep in mind that the diagnosis of AICA may be obscured by chronic otitis media without radiologic examination.In this report, clinical presentation, relevant radiologic findings and management of AICA were discussed with the literature knowledge.