In neuro-Behçet's disease nervous involvement may occasionally precede somatic manifestations, making the diagnosis challenging. The reported case showed only a neurologic onset with relapsing-remitting form, transient brain MRI abnormalities and late appearance of typical muco-cutaneous lesions, thereby delaying the correct diagnosis and appropriate treatment. MRI revealed cerebral alterations mimicking demyelinating disease, but lesions rapidly disappeared on close follow-up scans indicating vasogenic edema. Being aware of this atypical presentation and recognizing these transient abnormalities may be useful for a prompt diagnosis.