We retrospectively reviewed the imaging features of an aberrant course of the internal carotid artery (ICA) in one patient and its unilateral absence in four. Absence of the ICA was initially detected by MRI and MR angiography in both patients who underwent these examinations. CT revealed an abnormal or absent carotid canal in all cases. Radiological diagnosis by MRI and MR angiography could play an important role in the diagnosis.