An giant cell tumor arising from the tendon sheath and other soft tissue of the extremities, hasbeen of clinical and pathologic interst for many years, which is the second most common tumor of the hand, but it is not common in other location. One case of tendon sheath giant cell tumor with regional bone destruction on the pharangeal bone is presented which experienced at Department of Orthopaedic Surgery, Korea University recently. At first, the lesion of bone that is confused with Enchondroma in roentgenographically, which is confirned regional bone destruction of tendon sheath giant cell tumor by histologically. The tumor is composed of a vascularized network of spindle shaped or ovoid stromal cell regularly and rather interspersed with multinuclear cell and xanthoma cells. We report a case of giant cell tumor of tendon sheath and review of literature.