Leiomyosarcoma of the soft tissue involving bones is an extremely rare tumor. In the past, a hand tumor of this type required amputation. Presently, with improved chemotherapy and reconstruction, the hand and its dexterity sometimes can be saved. In this case, after chemotherapy, the authors opted for wide excision including the skin, tendons, muscles, and bones. The large defect was reconstructed with an iliac crest autograft and a dorsalis pedis free flap including tendons. Functional result was excellent in this patient. The pathologic results also were reviewed by histology, immunohistochemistry for muscle marker, and electron microscopy.