BACKGROUND Substantial progress has been made in the multimodality treatment of soft tissue sarcoma over the past decade. This review summarizes current state-of-the-art management for patients with extremity soft tissue sarcoma. METHODS Literature-based review of combined modality treatment of extremity sarcoma. RESULTS The revised staging system of the International Union Against Cancer/American Joint Committee on Cancer (UICC/AJCC) for this disease is described and a practical approach to diagnosis, evaluation, management, and follow-up of patients is presented. Brief summaries of the roles of surgical therapy, pre- and postoperative radiotherapy, pre- and postoperative chemotherapy, and concurrent chemoradiation in the management of extremity soft tissue sarcoma are provided, with summaries of the available data from randomized prospective trials. The data referenced and discussed herein provide the basis for stage-specific treatment recommendations for the management of patients with localized soft tissue sarcoma of the extremities. CONCLUSIONS Soft tissue sarcoma remains a challenging disease to treat. The majority of patients require an integrated combined modality approach. Future research should be directed at developing more effective systemic therapies and minimizing the toxicities of local therapies.