Corticobasal degeneration (CBD) is a rare neurodegenerative disease characterized by the predominance of pathological 4 repeat tau deposition in various cell types and anatomical regions. Corticobasal syndrome (CBS) is one of the clinical phenotypes associated with CBD pathology, manifesting as a progressive asymmetric akinetic-rigid, poorly levodopa-responsive parkinsonism, with cerebral cortical dysfunction. CBD can manifest as several clinical phenotypes, and similarly, CBS can also have a pathologic diagnosis other than CBD. This chapter discusses the clinical manifestations of pathologically confirmed CBD cases, the current diagnostic criteria, as well as the pathologic and neuroimaging findings of CBD/CBS. At present, therapeutic options for CBD remain symptomatic. Further research is needed to improve the clinical diagnosis of CBD, as well as studies on disease-modifying therapies for this relentlessly progressive neurodegenerative disorder.