A loss of speech can be related to disorders of the motor units (paresis), language deficits (aphasia), or speech programming deficits (apraxia of speech). Although apraxia of speech has been reported to be associated with degenerative diseases, we observed a patient with a unique constellation of signs that included apraxia of speech, oculo-orofacial apraxia and a supranuclear ophthalmoplegia in the absence of extrapyramidal (Parkinsonian) signs. Post-mortem examination revealed a loss of neurons in the frontal and temporal regions, but there was also a marked loss of neurons and astrogliosis in the caudate, claustrum, globus pallidus, substantia nigra, and loss of axons in the anterior cerebral peduncles. This patient's clinical presentation and the pathological correlates suggest that he might have suffered with a distinct disorder we call progressive oculo-orofacial-speech apraxia or POOSA.