Background: Recurrent ophthalmoplegic cranial neuropathy (ROCN), previously called ophthalmoplegic migraine, is characterized by recurrent episodes of headache and ophthalmoplegia of unclear etiology. Characteristic neuroimaging findings can support the diagnosis. Methods: A case report and review of the literature. Results: We present a 6-year-old girl with a past history of migraine headaches associated with retroorbital pain since 4 years of age. Family history is positive for migraine. She presented with a half a day history of left eye ptosis, 10 days post a resolved gastroenteritis which was associated with headaches. Examination showed only a left eye ptosis, pupil-sparing with no exotropia or diplopia. There was no headache. The rest of the neurologic examination was normal. Investigations showed normal blood tests and lumbar puncture. MRI head showed on thin cuts asymmetric nodular thickening (4mm) of the origin of the cisternal segment of the left oculomotor nerve, with corresponding homogeneous enhancement post gadolinium infusion. Clinical resolution occurred spontaneously within 48 hours. A review of the literature highlights focal thickening and enhancement of the affected cranial nerve, with resolution of enhancement post-acute phase. Conclusions: Focal thickening and enhancement of affected cranial nerve is seen in the majority of pediatric ROCN. These findings are best seen with thin MRI cuts and gadolinium infusion.