The oculomotor nerve of a 6-year-old boy with recurrent headache and recurrent ophthalmoplegia was contrast-enhanced on a magnetic resonance imaging (MRI) scan during an episode. The boy exhibited dramatic response to steroid treatment. The clinical features of ophthalmoplegic migraine and Tolosa-Hunt syndrome overlapped in this patient. We suggest that a positive MRI finding can be included as one of the diagnostic criteria in the classification of ophthalmoplegic migraine and that a trial of steroid is worthwhile in the presence of enhancement of the oculomotor nerve since ophthalmoplegic migraine may be noninfectious but inflammatory in etiology.