Introduction:Cortical basal ganglia degeneration (CBD) is currently considered to be a rare neurodegenerative disease characterized by pathological tau deposition, neuronal loss, ballooning, and glial degeneration in atrophic cerebral cortex and subcortical regions (including the substantia nigra and striatum) (1). On CBD magnetic resonance, the majority showed asymmetric cortical atrophy in the frontal, temporal, and parietal lobes of the brain. Typical MRI findings are asymmetric cortical atrophy in the premotor area, supplementary motor area and posterior cingulate gyrus, and middle frontal lobe (2).In this paper, we report a case of CBD misdiagnosed as Parkinson’s disease to summarize its clinical manifestations and imaging features, and have some experience in encountering such diseases in the future.