”Calcification of the nucleus accumbens in the basal ganglia, also known as idiopathic familial calcification of the basal ganglia (Fahr disease), is a rare neurodegenerative disease. The pathology of this disease is characterized by the presence of symmetrical calcium deposits and cell loss in specific brain regions (basal ganglia and cerebellum). The etiology is unclear, with most cases being genetically related, a few being disseminated, and occurring mainly in children. Clinical manifestations include spasticity, choreiform movements, and progressive intellectual decline. The patient has normal serum calcium and phosphorus. Small patches or masses of calcified spots in the paracranial region are seen on cranial plain films. CT scan of the skull has an important diagnostic value and shows symmetrical calcified spots in the bilateral basal ganglia (caudate nucleus, dentate nucleus), cerebellar dentate nucleus, thalamus, brainstem, hemi-oval center, and gray-white matter junction of the brain.