Primary hepatic leiomyosarcoma is a very rare tumor of the liver and primary hepatic leiomyosarcoma with Behcet's disease has not been reported previously. Behcet's disease is a multisystem disorder presenting with recurrent oral and genital ulcerations as well as ocular lesions; it has rarely been reported in association with malignant disease. We report a case of primary hepatic leiomyosarcoma with intrahepatic and abdominal subcutaneous metastasis in a patient with Behcet's disease; this is the first report of these findings in Korea.