Objective: Wilson's disease (WD) is a rare autosomal recessive syndrome due to a mutation of the ATP7B gene, characterized by Parkinsonian, hepatic, or psychiatric/cognitive presentation. Most WD cases present with symptoms between ages 12–23, with varying levels of cognitive deficit. Little is known about neuropsychological profiles in WD, as previous reports have used inconsistent, brief batteries, often administered post-treatment. We present a case of late-onset WD in its acute stage with severe cognitive impairment, motor symptoms, and personality changes. Method: “BV,” is a 63-year-old Caucasian man with 18 years of education, referred for assessment three months after receiving a diagnosis of WD. Cognitive complaints included problems with short-term memory/new learning, word finding, decision-making, perception, and writing, as well as episodes of disorientation. Motor symptoms included tremor, shuffling gait, and dyskinesas. Results: Testing revealed general intellect to be well below premorbid estimates. Deficits were seen across multiple domains, including processing speed, memory, executive functioning, and visuospatial abilities. Conversely, language remained intact. On self-report measures, BV denied affective distress or changes in frontal functioning. On the contrary, his wife reported significant changes in personality and frontal systems. Conclusion: This case aligns with studies identifying declines from baseline and frontal-subcortical patterns of deficit in WD patients. However, it differs in terms of the extent and severity of deficits typically reported. This case is also unique in its use of a comprehensive battery during the acute disease stage. Our findings suggest late-onset WD may result in greater cognitive, psychiatric, and motor symptoms than what is commonly associated with earlier onset.