Four examples of occipital condyle syndrome, that is, unilateral occipital pain and ipsilateral tongue paralysis due to selective erosion of the occipital condyle, are reported. The four patients complained of a continuous, severe, unilateral, occipital pain which kept them with the head rotated to the side of the pain and held with their hands. The pain became unbearable with head rotation to the nonpainful side and with unilateral suboccipital palpation. The onset of this very characteristic unilateral headache antedated by as long as 2 1/2 months the ipsilateral hypoglossal paralysis. The occipital condyle syndrome may be the first manifestation of cancer or of chronic inflammatory lesions; recognition of this distinctive headache enables the clinical diagnosis to be made.