本文报告肝豆状核变性18例,14岁以下9例,14岁以上9例。儿童的临床表现以慢性肝病为主,成人以神经精神症状为主。由于儿童的神经症状往往表现写字手发抖,吐词不清,行路不稳,常易被家长和医务人员认为是生长发育过程中的幼稚表现,造成漏诊,延误治疗。并对治疗中用药问题进行探讨. This article reports 18 cases of hepatolenticular degeneration, 9 cases under 14 years old, 9 cases over the age of 14. The clinical manifestations of children with chronic liver disease mainly adults with neuropsychiatric symptoms. Because children’s neurological symptoms often shuffle writing, unclear articulation and unsteadiness, they are often considered as a naive manifestation of growth and development by parents and medical personnel, resulting in missed diagnosis and delayed treatment. And treatment of medication problems to be explored.