Ophthalmoplegic migraine is a rare condition, previously thought to represent a variant of migraine. Recent observations regarding its usual clinical presentation and common magnetic resonance imaging findings have given rise to speculation that this illness is more likely to represent an inflammatory cranial neuropathy. The recent revision of the International Headache Classification has reclassified ophthalmoplegic migraine from a subtype of migraine to the category of neuralgia. In this article, potential pathophysiological mechanisms are discussed. The typical clinical presentation of ophthalmoplegic migraine generally involves transient migraine-like headache accompanied by often long-lasting oculomotor, abducens or, rarely, trochlear neuropathy with diplopia and (if oculomotor nerve is involved) pupillary abnormalities and ptosis. Ophthalmoplegic migraine generally occurs in children, but a number of adult cases have been reported. Prognosis is good because symptoms almost always resolve, but, after several episodes, some deficits may persist. Differential diagnosis is rather large, although most other possible causes of ophthalmoplegia and headache have distinctive presentations or can be excluded with fairly straightforward diagnostic testing. Optimal prophylactic and acute treatment is still unclear, but migraine prophylactic medications such as b blockers and calcium channel blockers have been proposed. Steroids have been used with mixed results.