Desmoid tumors are rare, benign fibroblastic tumors that are locally infiltrative and can cause extensive morbidity by destruction of adjacent vital structures. Due to the rarity of these tumors, evidence regarding optimal treatment protocols is drawn from case reports and single-arm series with small patient numbers. We report a case of a patient with a desmoid tumor of the left supraclavicular region that was diagnosed and treated in our department and a review of the current literature.