Progressive supranuclear palsy (PSP) is a heterogeneous progressive neurodegenerative disease characterized by onset after 50 years old, Parkinson’s syndrome, early development of postural instability, absence or transient reaction to levodopa drugs, neuropsychological disorders, dysphagia and dysarthria and eye movement disorders. The review provides an analysis of modern data on etiology, clinical presentation, differential diagnosis of the disease. The morphological picture and neuroimaging features, as well as modern ideas about treatment, are described. A great clinical polymorphism of the disease, as well as its similarity to other neurodegenerative diseases, manifested by Parkinson’s syndrome, complicates the diagnosis of PSP. Establishing an accurate diagnosis makes it possible to determine the prognosis and further tactics of patient management.