A 68-year-old male patient with Hallervorden-Spatz disease with autopsy findings is presented. He died 5 years after the occurrence of progressive rigidity, mask face, shuffling gait and tremor. Family history was negative. At autopsy, loss of neurons with gliosis in the globus pallidus and substantia nigra, large deposits of iron pigments and axonal spheroids in the same areas were found. So far as we know, this is the first case of Hallervorden-Spatz disease with autopsy findings reported in China.