Retroperitoneal sarcomas comprise a rare, heterogeneous group of neoplasms. Routine workup should include the patient's medical history, a physical examination, and helical CT of the abdomen and pelvis, with selective use of MRI and preoperative tissue sampling. The primary treatment is complete surgical resection with a rim of normal tissue. The role of adjuvant therapy is evolving and at present should not be used outside the investigational setting.