Painful ophthalmoplegia is characterized by unilateral involvement of the IIIrd, IVth and VIth cranial nerves, as well as supra- and retro-orbital pain, i.e. participation of the Vth cranial nerve. The pain is relieved within 48–72 h with steroid therapy. The paresis of the eye muscles in various combinations usually subsides gradually from within a few weeks to several months. The etiology is unknown. The few pathological examinations reported in the literature showed an unspecific inflammatory granulation tissue around the intracavernous portion of the carotid artery and on the dura mater in the vicinity of the cavernous sinus. Carotid arteriography may show stationary waves of this artery and narrowing of its intracavernous portion. With orbital phlebography the occlusion of the supraorbital vein and obstruction of the cavernous sinus are sometimes demonstrable. The syndrome is well defined and its etiology still unknown.