Primary progressive aphasia is a sub-group of frontotemporal dementia and a degenerative, rarely observed disease that only causes progressive deterioration in language functions for two years. Amyotrophic Lateral Sclerosis (ALS) is a progressive neuro-degenerative disease, clinically characterized with upper and lower motor neuron findings, accompanied by distinctive degeneration in upper and lower motor neurons of the spinal cord. Frontotemporal dementia together with ALS is a case stated in the literature. However, a faster progressing primary progressive aphasia, initiating with motor aphasia and with addition of motor neuron disease to the clinical situation, together with ALS was reported in recent years. Therefore, it was proposed to be assessed as a separate clinical entity. Both these diseases do not have an effective treatment yet. Our case is clinically initiating with primary progressive aphasia and two years later motor neuron disease started. The case is presented with a positron emission tomography (PET) data.