FTD is a group of neurodegenerative diseases with progressive deterioration of behavioral and speech disorders, morphologically associated with pathology of the frontal or temporal lobes. International clinical trials have made it possible to define modern diagnostic criteria for various subtypes of clinically «possible/probable» FTD. Our article is devoted to one of the rare subtypes of frontotemporal dementia (FTD), corticobasal syndrome (CBD), in which we presented a review of current data with a demonstration of clinical observation. A clinical case of a patient with a patient with speech disorders and memory impairment is presented. A 60-year-old man at the time of the outpatient visit had been complaining of speech impairment for two years, a slight decrease in memory for current events. Neurological and neuropsychological studies revealed two leading clinical syndromes in the patient: «frontal» syndrome with impaired higher cortical functions in the form of efferent motor aphasia, impaired writing and reading with visual-spatial agnosia and dysgraphia, «frontal» signs (positive «palm-mouth «and» grasping «reflexes); «Corticobasal syndrome» with pronounced dynamic, optic-kinesthetic dyspraxia, dermolexia, apraxia of closing the eyes, «alien» hand syndrome with symptoms of levitation and intermanual conflict. MRI diagnostics revealed changes characteristic of neurodegeneration of the frontotemporal type (atrophy of the frontal and temporal lobes prevails). Taking into account complaints, anamnesis of the disease, identified clinical syndromes and structural changes according to MRI data, the patient was diagnosed with a clinically «probable» FTD. Determination and accurate diagnosis of FTD subtypes will help the neurologist in managing these patients with the appointment of the correct pharmacologic treatment. In FTD, in contrast to AD patients, the administration of cholinesterase inhibitors does not lead to a positive therapeutic effect a positive therapeutic effect and, therefore, is not advisable. The standards of patient therapy should include recommendations for antipsychotic therapy, the use of antidepressants (SSRIs) and anxiolytics with nootropic effects for the correction of affective and behavioral disorders.