Radical surgical resection currently represents the most effective therapy for patients with retroperitoneal sarcoma. Unfortunately, margin-negative resection often mandates extirpation of multiple retroperitoneal viscera, and such operations are nonetheless fraught with high rates of locoregional recurrence. In an attempt to improve local control and ultimately survival, adjuvant strategies of radiation and chemotherapy have been increasingly employed, with promising results. To date, however, the rarity of the disease has limited large, prospective studies investigating the efficacy of these adjuvant modalities. In this article, we review the current literature pertaining to the diagnosis, staging and treatment of retroperitoneal sarcoma and demonstrate the critical need for future large, multi-institutional studies to advance our knowledge of this uncommon disease.