A 56-year-old Japanese male presented with tuberculous meningitis (TBM) manifesting as irrational behavior. He underwent lumbar puncture, which showed inflammatory cerebrospinal fluid (CSF) findings. Administration of anti-tuberculosis (TB) agents was started on the day after admission (Day 1) because delayed treatment of TBM might be fatal. On Day 4, magnetic resonance (MR) imaging and MR angiography showed fresh infarctions, hydrocephalus, and stenoses of arteries. CSF drainage and biopsy of brain tissue were performed, but the pathological findings were non-specific. Frequent CSF examinations, cultures, and polymerase chain reaction were performed, but no positive finding of TB was obtained. He died on Day 14. Brain autopsy showed Langerhans giant cells and Ziehl-Neelsen-positive TB bodies. Unfortunately, our patient suffered very poor outcome irrespective of early anti-TB treatment starting Day 1, suggesting the probability of delayed admission or drug-resistant TB. TB infection including TBM has become rare in developed countries, and diagnosis remains difficult. Corticosteroid therapy may be effective for TBM, but may be restricted and ameliorate mortality but not morbidity. Further study is required to establish second line treatment if TBM is resistant to anti-TB agents and corticosteroid administration.