Background: Fahr syndrome is a rare neuro-degenerative disorder characterized by symmetrical, bilateral calcifications in the basal ganglia, nucleus gyrus and cerebral cortex. It leads to cell loss in areas controlling movement and has build up of calcium concretions in the brain that can trigger epilepsy. Such intra-cranial calcifications are often but not always associated with chronic hypoparathyroidism and can have thyroid dysfunction too. Case Report: We present a case of 14 year old female presenting in status epilepticus previously been diagnosed as epilepsy, compliant with treatment for same since one year. Computed tomography head revealed diffuse cerebral calcifications in dentate nucleus with striato-pallidium calcifications. She was treated with intravenous calcium and antiepileptics and made remarkable recovery. Conclusion: This case highlights a unique and rare presentation of Fahr syndrome presenting with status epilepticus which subsided with treatment with intravenous calcium and antiepileptics.