We present here a case of Fahr’s disease in the pediatric age group. Fahr’s disease is a neurological, degenerative and rare disease, especially in this age group. It differs from Fahrs syndrome, which is associated with infectious pathologies, such as human immunodeficiency virus infection and metabolic causes, such as hypoparathyroidism. In contrast, Fahr’s disease has an idiopathic or familial cause and is related to neuropsychic symptoms. However, the differentiation of these terms is still poorly established in the literature. It has an unknown prevalence and affects individuals of both sexes in any age group, and individuals from the 4th decade are more likely to develop it. It has polygenic etiology being autosomal dominant, characterized by abnormal deposits of minerals, including mainly calcium and phosphate in the basal ganglia. It presents extra-pyramidal, psychiatric and epileptic manifestations. It is an incurable disease with progressive and irreversible evolution. Due to the involvement of the central nervous system, the prognosis is reserved and eventually fatal. The patient in question was MF, 15-years-old, male, with severe holocranial headache and convulsive crisis with findings of calcifications in the base ganglia bilaterally tomography of the skull.