Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare neuroinflammatory disorder that is typically associated with paraneoplastic and postinfectious processes. Opsoclonus myoclonus ataxia syndrome has not been previously reported in association with tuberculous meningitis (TBM). This report presents a unique case in which TBM manifested as OMAS, highlighting the complex interplay between tuberculosis and autoimmune neurological conditions. A 1.5-year-old previously healthy girl, presented with acute-onset jerky movements, opsoclonus, irritability, and reduced sleep over 4 weeks. A neurological examination revealed opsoclonus, irritability, generalized tremulousness, and fragmentary myoclonus. Neuroimaging was suggestive of TBM. A cerebrospinal fluid (CSF) analysis indicated lymphocytic pleocytosis with positive CSF cartridge-based nucleic acid amplification test results for tuberculosis. The patient was treated with methylprednisolone pulse therapy, intravenous immunoglobulins, and anti-tuberculous therapy (ATT). Significant symptom improvement was observed within 2 weeks. This case underscores a rare association between OMAS and TBM, demonstrating that tuberculosis can trigger OMAS through autoimmune mechanisms. A timely diagnosis and treatment with ATT and immunotherapy can lead to substantial recovery.