Objective To investigate the clinical manifestations,diagnosis and treatment of extensively drug-resistant tuberculosis (XDR-TB)meningitis. Methods One case of primary tuberculousis meningitis infected with multidrug-resistant mycobacteria was analyzed retrospectively.Relevant literatures were also reviewed by retrieving information through Wanfang Database and Pubmed using key words "multiple drug resistant tuberculosis meningitis","MDR tuberculosis meningitis","multiple drug resistant TBM","mul-drug resistant tuberculous meningitis","extensively drug resistant tuberculosis meningitis","XDR TBM","extensively drug resistant TBM" both in Chinese and English. Results A 24-year-old male patient,complained of headache,vomiting for 5 days,aggravated with mental abnormalities for 10 hours,with no history of pulmonary tuberculosis,was hospitalized in the Affiliated Hospital of Zunyi Medical College.The chest plain film was normal.Craniocerebral CT scan showed mild-hydrocephalus and cisterna ambiens stenosis.The patient died after undergoing anti-TB treatments with isoniazid(INH)0.3g iv qd,INH 0.3g po qd,rifampicin(RFP)0.45g qd,pyrazinamide(PZA)1.5g qd,ethambutol(EMB)0.75g qd,and dexamethasone(DEX)15mg qd.He was diagnosed as XDR-TB meningitis(as drug-resistant to isoniazid,rifampicin,streptomycin,ciprofloxacin,paminosalicylic acid,kanamycin,and protionamide ).Mycobacteria tuberculosis was isolated from his cerebrospinal fluid after 3 months.Five cases in 4 literatures were retrieved through Wanfang database and Pubmed among which 2 cases were initial treated,3 cases was unknown about initial treatment or re-treatment. Conclusions XDR-TB meningitis is rare in clinical practice with serious condition,rapid progress and high mortality rate.It is necessary to acquire drug susceptibility test results as soon as possible and adjust treatments according different conditions.A molecular drug susceptibility test may be helpful in the future.