Wilson’s disease is a rare autosomal recessive treatable disease of childhood or young adulthood (before 40 years). It results from abnormal functioning of an ATPase (ATP7B or WDNP) due to a mutation (more than 60 reported), insertion, or deletion of the ATP7B gene on chromosome 13q14.3-q21, responsible for a decreased or absent transport of copper into the bile and its accumulation in the organs, especially in the nervous system. The prognosis depends on the extension of hepatic and CNS lesions (astrocytosis and Opalski cells) before treatment instauration; if early enough, treatment allows to reverse non definite lesions. In case of irreversible lesions, treatment efficacy is limited and the quality of life of the patient is definitively compromised. Without treatment, the disease is fatal. Screening is possible by a low level of ceruloplasmine in the blood (less than 20 mg/dl), associated with a high copper level in the blood (generally more than 0.1 mg/l, 1.6 μmole/l) and increased cupruria (>0.10 mg/24 h). Dosage of hepatic copper by biopsy (>250 μg/g of dry tissue) or measurement of incorporation of marked copper may be necessary, but molecular biology is the most specific. In children, the disease generally starts with hepatic failure (60%), sometimes fulminating associated with acute hemolytic anemia, less frequently with dyskinesia or psychic disorders, while in adulthood it generally almost equally begins with a hepatic (50%), but also with neurologic or psychiatric features, such as dysarhria (55%), early onset parkinsonism (50%), atypical tremor or dyskinesias, especially dystonia and choreoathetosis (30%), loss of balance, gait disorder and ataxia (45%), changes in personality, character, depression or psychosis, the discovery of a corneal ring, loss of visual acuity, neuromuscular weakness, pyramidal signs or epilepsy. Forms presenting with hematologic, osteoarticular, endocrine, renal, gastro-intestinal, cardiac, dermatologic or other dysfunction are less frequent. The disease can involve the majority of organs. Brain CT or MRI show abnormal signal in basal ganglia, thalamus, brainstem, and white matter, reversible with early treatment by a copper chelator or an inhibitor of intestinal resorption such as penicillamine, trientine, Zn sulfates, tetramolybdate, but severe cases may require ultrafiltration or hepatic grafting.