Fahr's disease is a rare idiopathic bilateral and symmetrical calcification of the basal ganglia, thalami, subcortical hemispheric white matter and deep cerebellar nuclei. We report an unusual case of Fahr's disease in a 53-year-old man who presented with generalized seizure in our emergency department. Based on clinical, radiological and endocrinological appearance, the patient was diagnosed with Fahr's disease associated with hypoparathyroidism. Parenteral calcium and calcitriol supplementation were given in the emergency department. The clinical outcome was favorable after the treatment. Our case illustrates that Fahr's disease, though rarely seen, has to be considered in a patient with convulsive state associated with calcifications of the basal ganglia.