Purpose of Review <p>This review highlights the evolving management of desmoid tumors, including the importance of an accurate diagnosis, the potential role for surgery and radiation therapy in select cases, growing data supporting the use of radiology-guided techniques, as well as the emerging role of systemic therapy for disease not amenable to active surveillance.</p> Recent Findings <p>The initial management for desmoid tumors has shifted towards watchful waiting for a majority of patients. The recent FDA approval of nirogacestat, a gamma-secretase inhibitor, as well as prospective clinical trials examining multitargeted tyrosine kinase inhibitors, have increased systemic therapy options for patients. Local therapies, such as surgery, radiation and radiology-guided techniques, remain potential options in appropriate situations.</p> Summary <p>An improved understanding of the underlying biology and inherent behavior of desmoid tumors has resulted in a rapidly evolving and shifting treating paradigm. A multi-disciplinary approach is critically important to provide the best outcomes for patients.</p>