Objective: Primary progressive aphasia (PPA) is a rare neurodegenerative disease barely reported in China. Methods: Complete neuropsychological testing on memory, cognitive function and aphasia was carried out. The brain function and structure was also examined with MRI and PET imaging analysis. Results: The characteristics of clinical manifestation, neuropsychological testing and brain imaging analysis were reported. Except anomic aphasia, no other abnormal neuropsychological testing as well as sign of the nervous system was found. However, a significant atrophy and decrease in glucose metabolism of the left temporal lobe was observed. Conclusion: PPA is clinically characterized by progressive anomic aphasia without impairment of the cognitive function and other abnormal sign of central nervous system. Atrophy of the frontal and temporal lobe in the dominant hemisphere provides strong support for the diagnosis.