The incidence, pathophysiology, symptoms, differential diagnosis and treatment of ophthalmoplegic and retinal migraines are reviewed. We describe three cases: one recurrent, painful ophthalmoplegia alternating between the III and VI nerves, one recurrent migraine associated with internal ophthalmoplegia, and one migraine with visual aura and retinal infarction. In our review we emphasize their infrequency (ophthalmoplegic migraine, 0.7 per million; retinal migraine 0.5-7% of migraines with aura), their uncertain pathophysiology and the possibility of causing permanent oculomotor or visual deficits. Differential diagnoses, including all causes of painful ophthalmoplegia and amaurosis fugax are discussed, with special attention to the difficulty of differentiating Tolosa-Hunt syndrome and forms without headache.