Background <p>Fahr’s syndrome, characterized by bilateral symmetrical calcifications in the basal ganglia, thalamus, and cerebellum, is a rare disorder with diverse etiologies, including genetic, metabolic, and toxic. The disease’s pathogenesis remains incompletely understood.</p> Case presentation <p>Here, we present a case of Fahr’s syndrome secondary to primary hypoparathyroidism in a 55-year-old Indian female patient who presented with generalized tonic–clonic seizures, rigidity, speech difficulties, and altered consciousness. Neurological examination revealed positive Trousseau and Chvostek signs, and imaging showed bilateral calcifications in the basal ganglia, thalamus, cerebellum, and centrum semiovale. Laboratory tests confirmed hypocalcemia and low parathyroid hormone levels, leading to the diagnosis of hypocalcemic seizures due to hypoparathyroidism. The patient was treated with intravenous calcium, followed by oral calcium supplementation, calcifediol, and hydrochlorothiazide. Following treatment, she showed significant clinical improvement with the resolution of seizures, speech recovery, and reduced rigidity.</p> Conclusion <p>This case highlights the importance of considering Fahr’s syndrome in the differential diagnosis of patients with calcifications and neurological symptoms, particularly in the context of hypoparathyroidism. Early diagnosis and timely intervention can significantly improve clinical outcomes and prevent irreversible neurological damage. Further research is needed for the underlying mechanisms of calcification in Fahr’s syndrome and its clinical implications.</p>