Corticobasal degeneration is a progressive neurodegenerative disease that typically presents with asymmetrical parkinsonism and cognitive dysfunction. Recent molecular advances have given some clues to the pathogenesis of the disease. Clinical diagnosis is complicated by both the variability of presentation of true corticobasal degeneration, for example as a dementing illness, and the syndromes that look like it but are caused by other neurodegenerative diseases. Although definitive diagnosis of corticobasal degeneration can only be made at post-mortem examination, recent advances in imaging can assist the clinician with diagnosis. Treatment options remain limited and mostly address symptoms.