Copper-associated hepatitis is recognized with increasing frequency in dogs. The disease is characterized by centrolobular hepatic copper accumulation, leading to hepatitis and eventually cirrhosis. The only way to establish the diagnosis is by histologic assessment of copper distribution and copper quantification in a liver biopsy. Treatment with the copper chelator d-penicillamine is the most commonly used treatment. In addition, a low-copper/high-zinc diet can help prevent accumulation or reaccumulation of hepatic copper. Mutations in the copper metabolism genes COMMD1 or ATP7A and ATP7B have been associated with hepatic copper concentrations in Bedlington terriers and Labrador retrievers respectively. In the Labrador retriever, dietary copper intake contributes strongly to the disease phenotype.