Clinical Case Report: This paper presents the alterations and deficits in executive functions of a 33 years old man with Wilson's disease, patient at the Hospital Universitario La Samaritana in Bogotá, who in 2011 was diagnosed with cerebellar ataxia, and beginning to show a clinical picture of dysarthria and generalized motor difficulties. The presence of neuropsychological disorders, as well as the results obtained by Magnetic Resonance Imaging (MRI) of the brain and optical exam (Kayser-Fleischer rings) confirmed the diagnosis of Wilson's disease. Results and Conclusion: The neuropsychological profile of the patient showed alterations of attention and mnemonic processes associated with frontal functioning, as well as slowing-down of motor activities and low speed in processing information. The assessment of executive functions revealed impairment in cognitive flexibility, impulsivity and disinhibition, as well as difficulties in the process of planning, organizing and monitoring. All of these features indicated the presence of a dysexecutive syndrome in the patient and correlated with the results obtained by MRI.