This chapter presents the case of a 63-year-old right-handed woman who was referred for language difficulties. Significant deficits were present in executive and visuospatial functions, as well as language. Language was fluent, characterized by frequent semantic paraphasias, jargonophasia, and occasional word finding difficulties, and decreased understanding. The initial clinical diagnostic impression was that of a progressive but primarily focal cognitive disorder involving predominantly language: primary progressive aphasia (PPA). Autopsy confirmed the presence of a large left frontal hematoma with intraventricular spillage. Diffuse staining for b-amyloid was found in meningeal and cortical arteries, consistent with severe cerebral amyloid angiopathy (CAA). The differential diagnosis for PPA includes variants of fronto-temporal dementia (FTD) and atypical Alzheimer's disease (AD). The clinical profile, with the early onset, strong family history, absence of severe memory complaints on history and amnestic deficits on testing, behavioral changes, and a predominance of executive dysfunction, swayed the diagnosis towards FTD.