Background: Opsoclonus Myoclonus Ataxia Syndrome (OMAS), is an unusual autoimmune neurological manifestation. The common underlying triggers are either paraneoplastic or parainfectious while toxins and metabolite are less frequent. Neuroblastoma is the most common paraneoplastic condition in children. While in parainfectious causes variety of viral and bacterial association has been implicated. Case report: A 13-year-old male boy was presented with complaints of weakness, difficulty in standing and walking and abnormal eye movement from past 6 days. He had an episode of typhoid fever 15 days back and got relieved from acute enteritis symptoms within 10- 15 days but after that he developed all the classic symptoms of opsoclonus, myoclonus, ataxia, mood disturbances and reduced sleep within 2 – 3 days. His MRI brain, CSF analysis for oligoclonalband, EEG, PET scan whole body and VMA in urine were within normal limits except for Widal test. He was treated with IVIG and Dexamethasone. All his symptoms subsided fully after first cycle of immunosuppressive therapy . Conclusion: Although paraneoplastic condition is a common cause of OMAS in children butparainfectious causes are also important. Moreover, this case underscores that OMAS could be an immune mediated neurological complication of typhoid fever.