Abstract: Desmoid tumors (DTs) are rare intermediately malignant locally aggressive tumors that seldom cancerate and never metastasize. DTs are traditionally managed via radical resection. Throughout the years, disease management has evolved to utilize more conservative treatment strategies. This shift in treatment strategy does not only reflect the increased use of systemic treatment and the decreased use of resection and radiotherapy but also highlights the effectiveness of active surveillance as a first-line treatment strategy, replacing the previous concept of “diagnosis-then-treatment.” Novel drugs and treatment strategies are emerging, providing more treatment options for patients with DTs.