Introduction:Fahrs syndrome is a rare, slowly progressive, neurodegenerativedisorder, characterized by extensive, bilateral, and symmetrical basal ganglia calcification. It isassociatedwithneuropsychiatric manifestations and gradually progressive cognitive impairment. Fahrs syndrome is the secondaryform of brain calcification thatiscaused by variousmetabolic, infectious, or degenerativediseases. Case report:An old manwasadmittedto ourdepartmentwithrecurrentepisodes of syncope. An electrocardiogramrevealedcomplete AV block.Laboratoryresultsdisclosedhypocalcemia, pseudo hypoparathyroidism, and hyperphosphatemia. Computedtomographyrevealedintracranial calcifications suggestiveofFahrs syndrome. Conclusion:Fahrs syndrome is a rare anatomo-clinical and radiologicalentity, with multiple clinical and etiological aspects. The pathophysiologicalmechanism of the lesionsremainspoorlyunderstood, even more cardiacfeaturesthatmaycause, but is set to benefitfromadvances in functionalneuroimaging.