Progressive aphasia (PA) caused by neurodegenerative diseases is much less prevalent than aphasia following vascular brain lesions. Clinically, the progressive aphasias can be divided in progressive non-fluent aphasia, semantic dementia and logopenic aphasia. Differential diagnosis is based on a detailed language assessment and neuroimaging. Compared with the therapy of aphasia following stroke the treatment of patients with PA has been neglected. However, recently potential therapeutic options have been evaluated regarding feasibility and efficacy.