家族性基底节钙化症是一极少见的常染色体隐性遗传病 ,亦称Fahr氏病。笔者遇到 2例 ,系姐弟 ,报告如下。2例患者为亲姐弟 ,弟弟为先证者 ,因外伤入院治疗 ,CT检查发现本病。后邀其姐做CT检查亦证实患有本病。患者父母发育正常。例 1,男 ,14岁。体检 :神志清 ,身材矮小 ,身高仅 Familial basal ganglia calcification is an extremely rare autosomal recessive disease, also known as Fahr’s disease. The author encountered two cases, Department sister, the report is as follows. 2 patients were pro-siblings, younger brother proband, due to hospitalization for trauma, CT examination found the disease. Invited his sister to do CT examination also confirmed suffering from the disease. Patients with normal parental development. Example 1, male, 14 years old. Physical examination: clear mind, short stature, height only