Desmoids are a challenging group of lesions with variable biologic behavior and local morbidity but with no metastatic potential. Modern treatment involves a multidisciplinary and individualized approach based on the location, extent, and status of recurrence. Surgery continues to be a popular therapy for small resectable tumors. Radiation is also an effective alternative; however, its potential carcinogenic side effects must be weighed carefully in the context of young patients. Low-dose chemotherapy with methotrexate and vinblastine remains a noninvasive and safe treatment with a 10-year relapse-free rate of 70%. It is clear that desmoid tumors represent an extremely mixed population of tumors with very benign to very aggressive behavior. With better understanding of the molecular and genetic basis of this disease, it is hoped that more targeted therapies with minimal toxicity profiles will become available and become the preferred therapy.