Neurological manifestations present in 5% to 30% of patients with Behçet's disease. We studied consecutive patients with relapsing--remitting or progressive neuro-Behcet's disease who referred from January 2002 to January 2009 to Nemazee Hospital, Shiraz, southern Iran. Sequential MRIs were performed during clinical relapses in patients with relapsing--remitting course or during relentless progression after first referral of patients with progressive course. We reviewed 55 MRIs of 17 patients (ten men and seven women) with age of 36.4 ± 8.1 years at the time of first MRI. Nine (53%) patients had a relapsing-remitting course and eight (47%) had a progressive course. The initial and last follow-up studies had a mean interval of 29.2 months (range, 24 to 84). Of the patients with progressive neuro-Behcet's disease, 50% had brainstem atrophy and 75% had black holes in their last follow-up MRIs. The respective prevalence rates for those with relapsing--remitting neuro-Behcet's disease were 0% and 11%. In the total population of patients with neuro-Behcet's disease, the number of lesions (p = 0.002) and MRI burden (p = 0.016) had a significant increase in the last follow-up studies in comparison to the initial studies. Incremental pattern in the number of lesions and MRI burdens in patients with parenchymal neuro-Behcet's disease in our longitudinal study may imply an ongoing pathologic process.