Retroperitoneal sarcomas present a therapeutic challenge based on their location, extent of invasion at diagnosis, and propensity for local recurrence. Surgical therapy remains the only potentially curative treatment option; however, even with aggressive surgical approaches, local recurrence remains a common type of failure. For patients who have high-grade lesions, distant metastatic disease may also limit survival. Optimizing disease control while minimizing the morbidity of therapy remains the primary goal of management. In this article, the authors describe the presentation, evaluation, and management of patients who have retroperitoneal sarcoma.