Production of art is a complex process involving a combination of technical skill and a unique talent. Changes in artistic ability may accompany neurodegenerative disorders when they occur in an artist. The nature of these changes in the context of definable regional neuropathological disturbances may provide insight into the structural basis of the creative process. We describe a professional artist in whom presumed corticobasal degeneration (CBD) was associated with an alteration of his artistic judgement and production. Disinhibition, perseveration, and left hemispatial neglect, features of his cognitive profile were readily discernible in his work. The differences in his style are examined with respect to his main neuroanatomic abnormalities, namely right cerebral hemiatrophy as defined by magnetic resonance and single-photon emission computed tomography imaging. Cognitive deficits, including visuoconstructive and motor neglect, apathy, perseveration, and disinhibition as determined by neuropsychological testing, contributed to the dissolution of his artistic skills. Our case study adds to the growing literature on the effects of brain damage on artistic expression in the graphic arts.