Introduction: Fahr’s disease and Fahr’s syndrome represent clinical entities that result in diffuse intracranial brain calcification, either by way of genetic mutation in the case of the former or by secondary endocrine dysfunction in the latter.

Case Report: We present a case of a middle-aged male with undiagnosed Fahr’s syndrome, identified during evaluation for symptoms of an acute posterior circulation cerebrovascular accident.

Conclusion: Fahr’s syndrome is a clinical constellation of symptoms and radiographic findings often seen in late-stage hypoparathyroidism. The intracranial calcifications associated may be related to an increased risk for intracranial cerebrovascular disorders such as ischemic or hemorrhagic infarct.

Keywords: Fahr’s syndrome; case report; posterior circulation stroke; cerebellar infarct.