This article describes the aberrant course of the internal carotid artery (ICA) through the middle ear as an extremely rare vascular anomaly. On the basis of a case report, the findings of magnetic resonance imaging, computed tomography imaging, and especially color-coded duplex sonography are presented. The diagnosis of an aberrant ICA is important to avoid severe surgical complications of a retrotympanic mass as well as unnecessary diagnostic or therapeutic approaches in expectation of a stroke.