1. Introduction Fahr syndrome is characterized by bilateral calcifications in the basal ganglia, thalami, cerebellum, and cerebral subcortical white matter, which is primarily associated with endocrine abnormalities, particularly disturbances in parathyroid hormone (PTH) levels, such as hypoparathyroidism, and may be linked to brain infections or toxic exposures. Abnormal calcium deposition resulting from disorders in calcium and phosphate metabolism can lead to various neurological and psychiatric manifestations in young to middle-aged adults, including movement disorders, neuropsychiatric symptoms, and other central nervous system features. Epileptic seizures are infrequently reported in the literature, suggesting dysfunction in cortico-basal connections and interhemispheric interactions. The efficacy of anti-epileptic drugs for seizures in Fahr syndrome remains controversial. In this report, we present a case from our center and review cases from the literature who suffered from Fahr syndrome with epileptic seizures as a prominent symptom during the disease course.