The patient presenting with a red mass behind the eardrum and a pulsating tinnitus may well have a vascular tumor. One must be ever mindful, however, that the mass may represent a congential vascular anomaly. The most common one seen is the uncovered jugular bulb in which the vessel extends superiorly into the middle ear to or above the incudostapedial joint. More rare is the uncovered and posteriorly displaced carotid artery. The purpose of this paper is to review the literature on the subject of vascular anomalies of the middle ear and temporal bone and to discuss the diagnosis and management of these lesions. Illustrative case reports with color photographs are presented as well.