Wilson’s disease (WD) is a rare autosomal recessive disorder of copper metabolism, also known as hepatolenticular degeneration. It is caused by mutations in the ATPase copper binding protein beta (ATP7B) gene, resulting in toxic levels of copper accumulation in the liver, cornea, central nervous system, and kidneys. This chapter focuses on the clinical features and laboratory findings of WD, as well as the various histologic patterns that can manifest as other liver diseases. The diagnosis of WD cannot be made by pathologic examination alone and requires clinicopathologic correlation. The first step in making the diagnosis and starting the work-up is to consider WD in the differential diagnosis. Our responsibility as pathologists is to be familiar with the clinical and histological findings of WD and to raise this concern for every patient with unexplained liver enzyme elevations or pathology.