Publisher Summary Calcification of the basal ganglia (BG) occurs in many contexts, ranging from incidental findings to disorders of mineral metabolism, and familial forms of cerebral calcinosis. Bilateral striopallidodentate calcinosis (BSPDC), commonly known as Fahr's disease is an idiopathic, familial form of calcification of the BG presented as a combination of Parkinsonism, dementia, and cerebellar signs. Computed tomography (CT) is significantly used for detecting BG calcifications over skull plain films. A subset of BG calcifications exists for which there are identifiable metabolic or degenerative causes. In contrast to incidental cases without a known etiology, these symptomatic calcifications are often associated with a progressive syndrome of Parkinsonism, dystonia, chorea, and tremor. The conditions associated with symptomatic BG calcifications can be essentially classified into idiopathic or secondary causes. BSPDC is characterized with varying symptoms of dementia, dysarthria, Parkinsonism, pyramidal, and cerebellar signs. Onset of symptoms typically occurs between 30 and 50 years, and cognitive symptoms are not uncommon. The cognitive aspects of BSPDC are well documented and supported by frontal–subcortical circuits.