Corticobasal degeneration (CBD) is a rare neurodegenerative disorder. The most common presentation of CBD is the corticobasal syndrome (CBS), which is a constellation of cortical and extrapyramidal symptoms and signs. Clinical-pathological studies have illustrated that CBD can present with diverse clinical phenotypes, including a non-fluent, agrammatic primary progressive aphasia syndrome, a behavioral, dysexecutive and visuospatial syndrome, as well as a progressive supranuclear palsy-like syndrome. Conversely, multiple pathologies, such as CBD, Alzheimer's disease and progressive supranuclear palsy may underlie a patient with CBS. This clinical-pathological overlap emphasizes the need for biomarkers that will assist in the accurate diagnosis of patients with CBS. This review presents an overview of the pathological, genetic, clinical and therapeutic characteristics of CBD, with an emphasis on the imaging (structural and functional) and biochemical (cerebrospinal fluid) biomarkers of CBD.