Introduction: The Behçet\'s disease with neurological involvement is recognized as Neuro-Behçet\'s syndrome and it is one of the most devastating manifestations of Behçet\'s disease. It involves central nervous system and has significant impacts on patient’s prognosis and quality of life. According to recent reports, central nervous system involvements are found in 5% of patients of Behçet\'s disease in Korea. Neuro-Behçet\'s syndrome is characterized in most cases by the presence of aseptic meningitis, cerebellar signs, intracranial hypertensions, and pyramidal alterations. Miliary tuberculosis(TB) is a potentially lethal disease if not diagnosed and treated early. Miliary TB is more commonly seen in immunosuppressed individuals but miliary TB cases related or superimposed on Behcet’s disease are not found commonly. Case: A-14–year old patient with Behçet\'s disease who had been treated with colchicine for more than 1 year, presented to emergency center due to fever, headache, and vomiting. Lumbar puncture was performed at the time of admission. Cerebrospinal fluid analysis revealed 83 leucocytes/ml (100% were lymphocyte), protein and sugar were 128.5mg/dl and 40 mg/dl respectively. Chest X-ray and chest high resolution computed tomography findings demonstrated wide spread of small nodular opacities distributed through both lungs. Given the above findings, we arrived at a diagnosis of TB meningitis with pulmonary miliary TB. A four drug regimen: isoniazid, pyrazinamide, rifampicin, ethambutol and intravenous dexamethasone were applied as an initial treatment. Throughout history taking, we found that she had taken colchicine for her Behçet\'s disease for 15 months and had 4 months of oral steroid therapy which was stopped about 1 year ago. Brain magnetic resonance imaging was performed and it demonstrated a diffuse pial enhancement in brainstem, multifocal hyperintense lesions on T2 weighted images in the bilateral centrum semiovale and right parietotemporal whitematter. A final diagnosis was changed from TB meningitis to Neuro-Behçet\'s syndrome with miliary TB. After primary treatment, her condition stabilized and she was discharged from the hospital 19 days later. Conclusion: We report a case of Neuro-Behçet\'s syndrome presenting with features of acute meningitis with miliary tuberculosis