The review is focused on essential tremor (ET), the most common extrapyramidal system disorder. Current understanding of the disease pathogenesis is provided; issues of classification and differential diagnosis are discussed. Modern ET treatment methods include therapeutic approaches and surgical interventions. The benefits of the new ET treatment method, the magnetic resonance-guided focused ultrasound treatment (MRgFUS), are described; the world’s experience of using the method, indications and contraindications are summarized.