A debate has been going on for many years about whether migraine with aura and migraine without aura are part of the same disorder or should instead be considered as two separate disorders. Although no final consensus has yet been reached on this issue, many clinical and pathogenetic elements suggest that the second option is true. Clinically, migraine with aura and migraine without aura are differentiated by epidemiological features, the characteristics of the headache phase, patient behaviour during attacks, natural history, age at onset and age at resolution, the recurrence pattern of attacks, favouring circumstances and trigger factors, correlation to female reproductive events, comorbidity and preventive therapies. Moreover, several literature reports suggest a possible different pathogenetic basis for the two forms of migraine.