Language disturbances are common features occurring in different neurodegenerative diseases, including Alzheimer's disease (AD) and the Frontotemporal Lobar Degeneration (FTLD) variants Primary Progressive Aphasia (PPA) and Semantic Dementia (SD). Despite AD and FTLD are supposed to have a different pathophysiology, PPA has been demonstrated to have in some cases an AD pathological component. The syndromic and etiological heterogeneity is crucial for the differential diagnosis and consequently for a therapeutical approach. Here, the case of a patient with progressive isolated language disturbances is presented, and further discussed on the basis of current diagnostic criteria and available guidelines for treatment.