Fahr disease is a rare neurological condition characterized by abnormal basal ganglia calcification. Some patients with Fahr disease have been reported to present to the emergency department (ED) with stroke-like symptoms. Here, we present a case of Fahr disease with transient focal neurological signs. A 63-year-old man came to the ED with complaints of slurred speech and abnormal movement of his left arm. His symptoms lasted only 5 minutes before he recovered. He had no focal neurological deficits when he arrived at the ED, had no previous medical history and had never taken any medication. A physical examination revealed that the patient was healthy, and the initial results of laboratory tests were all within normal range. A brain computed tomography (CT) without contrast enhancement revealed symmetric calcification of the basal ganglia, thalamus and dentate nucleus of the cerebellum. Magnetic resonance imaging of the brain and neck CT angiography revealed no evidence of acute cerebral infarction, intracranial large-vessel occlusion, or stenosis. Based on his clinical presentation and examination results, the patient was diagnosed with Fahr disease in the ED. He was recommended to be admitted for differential diagnosis of seizures and additional tests, such as electroencephalography, but opted for discharge against medical advice. Emergency physicians should consider the possibility of Fahr disease if patients have focal neurological signs with characteristic symmetric calcification on brain CT. A detailed history and examination should be performed to detect other secondary causes associated with Fahr syndrome.