Behcet's disease (BD) is characterized by recurrent oral and genital ulcers and uveitis. It is also known to affect various organs; however, central nervous system involvement is rare and may cause parenchymal or vascular lesions with distinct clinical syndromes. Various viral encephalitides are important differential diagnoses. MRI may aid the correct diagnosis. This case report describes a patient with acute severe neurological involvement due to neuro-BD.