A man of 61 with a 26-yr history of progressive cerebellar ataxia was admitted to the hospital. He had chronic liver disease and died 22 days after admission. A diagnosis of hepatolenticular degeneration (Wilson''s disease) was supported by clinical investigations and confirmed at autopsy, when tissue Cu studies were performed. Several unusual features were present, including a unilateral Kayser-Fleischer ring, a hepatocellular carcinoma, peripheral neuropathy, pontine demyelination and calcification of neurons in the medulla. The significance of these findings is discussed with a review of the relevant literature.