Summary <p>Retroperitoneal sarcomas (RPS) are a group of rare malignancies with many histologic types. Surgery is the hallmark of curative therapy for non-metastatic disease with the extent of resection depending on the histology.</p> Recent Findings <p>Neoadjuvant therapies have limited response and efficacy in RPS; however, the benefit, regimen, and timing depend on histology. Innovative therapeutics are emerging and may prove promising.</p> Purpose of Review <p>This review discusses the work-up, management, and advances in the field of retroperitoneal sarcoma.</p>