Corticobasal degeneration is one of the neurodegenerative tauopathies, which are defined as a group of heterogeneous dementias and movement disorders that are characterized neuropathologically by prominent intracellular accumulations of abnormal filaments formed by the microtubule-associated protein tau. Although there are no curative treatments, symptomatic and supportive management can be helpful. Many new therapies are still under development. However, more needs to be learned about the pathogenesis and molecular biology of this disease before an effective therapy can be developed.