Wilson Disease is kind of an autosomal recessive genetic disease. Early diagnosis and timely treatment are very important for prognosis. This article reviews the treatment of Wilson Disease, focusing on penicillamine, sodium dimercaptopropane sulfonate, ammonium tetrathiomolybdate and zinc, liver transplantation and gene therapy. At the same time, the problems of medication adherence and follow-up evaluation in patients with Wilson Disease are also discussed.肝豆状核变性是一种常染色体隐性遗传病,早期诊断和及时治疗对预后至关重要。现就肝豆状核变性的治疗现状进行综述与展望,重点介绍青霉胺、二巯基丙磺酸钠、四硫代钼酸铵和锌剂等药物治疗、肝移植以及基因疗法的现状及进展,同时也讨论了肝豆状核变性患者用药依从性和随访评估等方面的问题。.