Apraxia of speech (AOS) can be caused by neurodegenerative disease and sometimes is its presenting sign (i.e., primary progressive apraxia of speech, PPAOS). During the last several decades our understanding of PPAOS has evolved from clinical recognition to a fuller understanding of its core and associated clinical features, its distinction from but relationship with primary progressive aphasia, its temporal course and eventual progression to include other neurological deficits, and its neuroimaging correlates and underlying pathology.This paper provides a comprehensive summary of the literature that has built the current knowledge base about PPAOS and progressive AOS as it co-occurs with progressive aphasia. It reviews the history of its emergence as a recognized syndrome; its relationship with the agrammatic/nonfluent variant of primary progressive aphasia; its salient perceptual features and subtypes; the acoustic and structural/physiological imaging measures that index its presence, severity, and distinction from aphasia; and principles and available data regarding its management and care.A broad summary of what is known about AOS as a manifestation of neurodegenerative disease.Primary progressive apraxia of speech is a recognizable syndrome that can be distinguished from other neurodegenerative conditions that affect speech and language.