Introduction. Fahr?s syndrome is a rare, slowly progressive,
neurodegenerative disorder, characterised by extensive, bilateral, and
symmetrical basal ganglia calcification. It is associated with
neuropsychiatric manifestations and gradually progressive cognitive
impairment. Fahr's syndrome is the secondary form of brain calcification that
is caused by various metabolic, infectious, or degenerative diseases. Case
report. We presented a middle-aged male with Fahr's syndrome due to primary
idiopathic hypoparathyroidism. Clinical diagnosis was based on signs and
symptoms of hypocalcemia, progressive neuropsychiatric illnesses, laboratory
evidence of hypoparathyroidism, and radiological signs of calcifications in
the basal ganglia. The patient improved after only a few days of intravenous
rehydration and calcium substitution, followed by oral supplemental
calcitriol. Conclusion. Timely recognition of idiopathic and iatrogenic
hypoparathyroidism allows appropriate treatment that can prevent the
development and clinical manifestations of Fahr?s syndrome and potentially
slow its progression.