Abstract: Desmoid tumors (DT) are rare, locally aggressive tumors that lack metastatic ability but tend to grow invasively and recur locally. This can severely impair patient function, thereby causing significant clinical burden and even death. Over the past 20 years, there has been a significant shift in the treatment philosophy for this disease. Conservative methods with active surveillance are being employed as the first-line of treatment as opposed to aggressive surgeries that were previously the norm. The range of treatment options has also expanded, revealing a trend towards multidisciplinary management. Studies have indicated the pathogenesis of DT to be linked to the dysregulation of Wnt and Notch signaling pathways. Several new drugs targeting these pathways, such as nirogacestat, are currently under development and are expected to be a part of future treatments for this disease. This article reviews the molecular pathogenesis and advances in the treatment of DT, providing a basis and direction for clinical treatment and future research on this disease.