Abstract: A case of Hallervorden-Spatz disease with autopsy findings in a 68-year-old man is presented. The patient died after 5 years of progressive rigidity, mask face, shuffling gait and tremor. NO family history was available. At autopsy, there was loss of neurons with gliosis in the globus pallidus and axonal spheroids were found in the same areas. This is the first case of Hallervorden-Spatz disease with autopsy fings reported in China.