The review article outlines modern aspects of the nomenclature, etiopathogenesis, diagnosis and treatment of tenosynovial giant cell tumor of the tendon sheath. This is the second most common benign neoplasm of the hand. Non-radical surgical treatment of this disease leads to a large number of recurrence. To avoid tumor recurrence, surgeon must to have the basics of plastic reconstructive surgery, use delicate instruments and optical magnification. In some cases, adjuvant radiation therapy and administration of tyrosine kinase inhibitors may be required.