Desmoid tumors are benign tumors that exhibit varying degrees of local aggressiveness and diverse growth patterns. Magnetic resonance imaging remains the modality of choice for assessment of the nature and size of the soft-tissue lesion and involvement of surrounding structures. Treatment strategies include surgery, chemotherapy, hormonal therapy, and radiation therapy, either individually or in combination. Despite the benign nature of these tumors, multidisciplinary care is needed to provide combined treatment options. Chemotherapy in low doses is an excellent first-round treatment in any patient in whom contemplated local treatment may produce local morbidity and adjacent tissue injury.

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