We report the case of a 34-year-old man with pulsatile tinnitus and a reddish mass in the anteroinferior quadrant of the middle ear. Physical examination and imaging were unable to establish a diagnosis, so an exploratory tympanotomy was performed. Exploration revealed the presence of an ectatic aberrant internal carotid artery in the middle ear. Aberrations of the internal carotid artery in the middle ear are rare. Even so, our case is unusual in that all initial investigations had failed to establish the diagnosis. This case highlights the limitations of modern imaging techniques in certain situations.