© 2018 Wolters Kluwer Health, Inc. All rights reserved. Mixed transcortical aphasia (MTA) is characterized by decreased spontaneous speech, impaired naming, and poor comprehension, but with intact repetition. MTA has been reported to be the sequela of left hemisphere watershed infarction that isolates Wernicke's perisylvian arc. We report a 55-year-old right-handed woman who began having word-finding difficulty and then gradually developed impaired spontaneous speech, comprehension, and naming, but with intact repetition. Magnetic resonance imaging showed atrophy in the left frontal, parietal, and temporal lobes. This patient demonstrates that MTA can occur as a result of neurodegenerative disease. Further research is needed to learn whether progressive MTA is a late stage of primary progressive aphasia, examine the neuropathology associated with this syndrome, and identify treatment strategies.