This is one of the treatable conditions among all cases with movement disorders, if diagnosed early. All possible measures should be undertaken to diagnose this condition since it may have varied presentations. There is far reaching advancement in molecular pathogenesis and diagnosis of Wilson disease; still we consider that the present state of molecular-biology can not explain the variable clinical presentations. There is a great role of neuroimaging diagnostically and therapeutically. Treatment options are very limited, since in many countries the recently acclaimed safer agent such as tetrathiomolybdate is not available. We have got wide experience of using drug such as d-penicillamine and hopefully in future those safer agents will be available at affordable prices. In this chapter we have comprehensively dealt with all aspects of Wilson disease.