Wilson’s disease is an autosomal recessive disorder of hepatic copper disposition caused by mutations in the gene ATP7B, located on chromosome 13. This gene encodes a P-type ATPase, known as the Wilson ATPase, which functions within hepatocytes to move copper across intracellular membranes. The copper-transporting action directly supports production of the ferroxidase ceruloplasmin, in which copper is incorporated, as well as excretion of copper into bile. Consequently, in Wilson’s disease, serum concentrations of copper are low and hepatic retention of copper develops, leading to liver injury. Wilson’s disease can present as hepatic, neurological or psychiatric disease; clinical phenotypes are highly varied. Other organ systems may also be involved. Although the usual age range for clinical presentation is 5−45 years, younger children and older adults may present with this disease. Clinical investigations include tests of liver function, cerebral imaging, serum ceruloplasmin and copper, basal 24-hour urinary copper measurement, and hepatic parenchymal copper concentration. Genetic diagnosis is complex and best used for family studies or in populations known to have a limited repertoire of mutations. Treatment is usually very effective and consists of life-long administration of a chelator (D-penicillamine or trientine) or of zinc in pharmacological doses. Liver transplantation is mandatory for patients presenting with fulminant hepatic failure (coagulopathy, encephalopathy, massive intravascular haemolysis, renal failure, elevated aminotransferases, subnormal alkaline phosphatase) or for those whose liver disease is not responding to usual treatment. Liver cancer is extremely uncommon in patients with Wilson’s disease but screening may be appropriate for older patients.