Wilson disease is an autosomal recessive inherited disorder of human copper metabolism clinically associated with hepatic damage and/or neurological symptoms of varying degree. Copper accumulation and toxicity result in direct injury to hepatocytes followed by inflammation and irreversible impairment of neurons, mainly in the extrapyramidal system. A not insignificant number of cases begin with fulminant liver failure or acute appearance of neurological symptoms. If left untreated or in the case of delayed diagnosis and treatment, both acute manifestations may result in irreversible symptoms or even death. Rapid and exact diagnosis by means of clinical, biochemical and genetic analysis and the immediate initiation of drug therapy with copper chelators or, in the case of fulminant liver failure, orthotopic liver transplantation are essential for a favourable outcome in patients with acute Wilson disease.