Author- Dr Shikha Rowlak,Senior resident, Bombay Hospital. Title-Low grade glioma with atypical clinical and radiological presentation. Keywords-Paroxysmal sympathetic hyperactivity,(LGG)low grade glioma,(DNET)dysembryoplastic neuroepithelial tumor. Statement of the problem Paroxysmal sympathetic hyperactivity (PSH)can be a presentation of low grade glioma which subsides automatically after excision of the tumor. Paroxysmal sympathetic hyperactivity (PSH) is a complication of severe traumatic or hypoxic brain injury, characterised by transient episodes of tachycardia, tachypnea, hypertension, hyperthermia, diaphoresis, and/or dystonic posturing.They are associated with poor outcomes. PSH rarely occurs after brain tumor resection in pediatric patients; only 4 cases have been published since 1929.  Methodology and theoretical orientation A 18 years old, right handed male, came with slurring of speech with heaviness of tongue, Left eye ptosis and Imbalance while walking since 2 days. He was operated for meningimyelocele and Spina bifida when he was 3 days old after that he developed urinary incontinence and requires enema for motions. He was also operated for left parieto occipital SOL at the age of 10 years and Histopathology was suggestive of DNET/low grade glioma.In 2007 he had subacute vascular insult in left parieto occipital region.The patient on evaluation was hypertensive and tachycardic.Conscious, oriented with Pupils : Right 1.5 mm Left 4mm both reactive to light. Extraoccular movements : Restricted in all directions in both eyes except R eye abduction present. Speech was slurred. Fasciculations present on Right side of tongue which was atrophied and deviated to right and uvula was deviated to left. Tone : Increased on right side.Balance was impaired. Finding  Completed MRI suggestive of Post operative changes in the left temporo occipital with significant progression of residual / recurrent disease with spread along the ependymal lining of the lateral ventricles with mild dilatation. Raised intracranial pressure.Pial enhancement raised a possibility of Leptomeningeal disease / Meningitis.During the course of admission he has been continuously hypertensive, tachycardic and febrile which were all managed conservatively for few days. CSF analysis ruled out infection. Craniotomy and excision of the recurrent lesion was done along with V P Shunt on 6th day from admission. Histopathology report- pilocytic astrocytoma WHO Grade 1.Patient has been recovering. Conclusion and significance PSH patients are usually younger, tended to have more aggressive, higher-grade tumors located in deep brain regions in or around the ventricles.Such delayed presentation in a low grade tumor that recurred is rare.It requires prolonged hospital stay and can lead to  poor outcomes. Disclosure of conflict of interest -None.