A case with unilateral hypoplasia of the internal carotid artery was reported. The rerouting of anomalous vessels in the middle ear, visualized by otoscopy, was demonstrated with CT and MRI. Associations with internal carotid hypoplasia, the intercarotid collaterals, both anomalous middle ear arteries and the enlarged internal maxillary artery were confirmed with MRI. MR images were helpful to diagnose not only the anomalous vessels but also the multiple anastomoses.