Intratympanically aberrant internal carotid artery(ICA) is a rarely seen vascular abnormality. We present here the combination of aberration and prominent hypoplasia of the (CA in a case. Intratympanic aberrant ICA, which is rarely cited as a cause of tinnitus and hearing loss, should be known as a reason to be kept in mind as it may lead to life-threatening complications. Generally, it has been defined upon massive bleeding during myringotomy, ear surgery or biopsy procedure. In this article, the audiological and radiological studies confirmed with CT and MR angiography conducted on an aberrant and hypoplastic internal carotid artery that was identified under the manubrium mallei in a 28-year-old, young male patient who presented with complaints about hearing loss and fullness in the left ear were presented along with a literature review. (C) 2013 Elsevier Inc. All rights reserved.