A 53-year-old woman visited us for a neurological consultation before her liver transplantation. She had a history of primary biliary cirrhosis that began at 37 years of age. She showed falling episodes and met with a traffic accident at 52 years old. Since then, her symptoms had worsened. The neurological examination showed masked face, rigidity on bilateral arms and legs, and lack of balance. Her Mini-Mental State Examination Score was 28/30, but she suffered from loss of memory and had trouble with executive function in detailed examinations. Her T(1) weighted image showed hyperintensity in bilateral globus pallidus, putamen, dentate nucleus and cerebral peduncle. There was a significant improvement in intellectual function and neurological signs 6 months after her orthotopic liver transplantation. In addition, post-liver transplantation images showed a decrease in the area of hyperintensities. This case suggests that even in a patient with severe liver cirrhosis a complete cure of neurological manifestations can be obtained after the liver transplantation.