Wilson's disease (hepatolenticular degeneration, dystrophia hepatocerebralis)--a chronic, progressive disease with a genetically determined autosomal recessive mode of inheritance. The violence in metabolism of copper with its excessive accumulation in visceral organs and central nervous system is the basis of the disease. The guidelines on clinical diagnosis and management of patients based on a Review and analysis of recent publications on this topic in the global and Domestic literature and on the authors' experience in treating patients with Wilson's disease.