In this rare variety of complicated migraine the headaches are associated with ocular motor nerve palsies. Usually the ophthalmoplegia is transient; however, it can become permanent especially after repeated attacks. In the differential diagnosis, consideration should be given to aneurysm, tumor, diabetes, and sphenoid sinus mucocele. The age at onset, negative glucose tolerance test, and radiologic studies will usually rule out the listed possibilities. Other clinical entities confused with ophthalmoplegic migraine have included myasthenia gravis and the Tolosa-Hunt syndrome. The former condition is ruled out if the pupil is involved (and actually should not be considered in the presence of pain) and with response to edrophonium chloride (Tensilon); the latter possibility should be considered if pain persists. On rare occasions only limited involvement of the third nerve occurs. In the differential diagnosis, suspicion would be raised by 1) the absence of a migraine history; 2) severe persistent headache with total ophthalmoplegia; 3) onset after age 20; and 4) symptoms and signs of subarachnoid hemorrhage. Angiography is not warranted in a young patient strictly fulling the clinical criteria. Now, however, this is a diagnosis of exclusion, and noninvasive imaging tests such as magnetic resonance imaging (MRI) or magnetic resonance angiography (MRA) should be performed in all cases to exclude the possibility of aneurysm. Ideally, prophylactic therapy would prevent the occurrence of repeated episodes and prevent the development of permanent eye muscle palsies, but reports suggest that therapy has met with only limited success. A trial with calcium channel blocking drugs such as verapamil or beta blocking drugs such as propranolol or even methysergide may be warranted if the attacks are frequent.