The aim of the paper was to draw attention to a special group of patients with dementia who develop dominant impairment of language capabilities within the first two years of disease. We present a disorder known as primary progressive aphasia (PPA). The paper describes subtypes, symptoms and diagnostic criteria for each of the subtypes, along with therapeutic guidelines. Based on this case report, we present diagnostic and therapeutic difficulties likely to be encountered by doctors working with this rare patient population. The effects of depressive symptoms blurring the clinical picture are emphasised. The role of neuropsychological assessment and neuroimaging with the choice of magnetic resonance imaging due to the limited access to SPECT and PET, and lower utility of commonly used computed tomography is also emphasised. Limited therapeutic options for patients with PPA belonging to the frontotemporal dementia spectrum, as well as the chance to delay the progression of the disorder in language-variant Alzheimer's disease with the use of pharmacotherapy are also described.