A 41-year-old male presented with a four-day history of fever, recurrent oral ulcers, and a relapsing protruding rash on his neck. He complained of impaired speech fluency, somasthenia, headache, and unsteady gaits. Neurological examination revealed dysarthria, hyperreflexia in the right upper limb, and bilateral dorsiflexion Babinski sign. No retinal or conjunctival abnormalities, genital ulcers, and leprosy-skin lesions were found. Pathergy test was positive. Laboratory tests reported leukocytosis (12.95x109/L) with neutrophils predominating and elevated CRP (33.3 mg/L) in blood, while CSF analysis showed lymphocytic pleocytosis. Antibodies testing including NMDAR, AMPA1/2, AQP4, MOG, GAD65, and LGI1 were negative. The MRI with contrast showed hypointensity on T1 and hyperintensity on T2 and T2-FLAIR in the bilateral frontal or temporal lobe and corona radiata, as well as in the left caudate nucleus and left parietal lobe. Intracranial Doppler flow diagram showed an increased blood flow velocity in anterior cerebral artery and middle cerebral artery, with an increased cerebral vascular pulsatility index, suggesting stenosis of the cerebral artery. A diagnosis of NBD was made based on the characteristic cerebrovascular inflammation, indicated by elevated neutrophil levels, Doppler flow diagram findings, and clinical findings including oral ulcer, relapsing rash, and positive pathergy test result. The patient was treated with high-dose corticosteroids and intravenous immunoglobulins and experienced a rapid resolution of the rash and ulcers, an improvement of headache and gait, and regression of MRI lesions.