Hallervordren-Spatz病(Hallervorden Spatz disease,HSD)又称苍白球黑质色素变性病,是一种罕见的常染色体隐性遗传病,是由于铁代谢障碍导致Fe3+在苍白球、黑质、红核等部位内沉积所引起的一种罕见的中枢神经系统变性病,具有很强的临床和遗传异质性。本研究报道1例本院发现的临床诊断HSD的病例,并复习相关文献。1病例 Hallervordren-Spatz disease (Hallervorden Spatz disease, HSD), also known as globus pallidus degeneration, is a rare autosomal recessive disease is due to iron metabolism disorders lead to Fe3 in globus pallidus, substantia nigra, red nucleus, etc. A rare central nervous system degenerative disease caused by deposits within the site, with strong clinical and genetic heterogeneity. This study reports a case of clinical diagnosis of HSD found in our hospital, and review the relevant literature. 1 case