The aim of this chapter is to focus on the corticobasal degeneration. In 1968, corticobasal degeneration (CBD) was first described as a distinct clinicopathological entity by Rebeiz et al. Corticobasal syndrome (CBS) was initially described as the pathognomonic clinical presentation of CBD. This chapter covers the different clinical phenotypes associated with corticobasal degeneration pathology, neuropathology and clinicopathological correlations, epidemiology and natural history, clinical presentations, clinical diagnostic criteria and validation, clinical diagnostic approach and investigations, pitfalls in clinical diagnosis, genetics, and treatment. Treatment in CBD and related phenotypes is mainly symptomatic. Evidence-based trials are limited and treatment options suggested in this section are based on anecdotal experience.