A 66 years old female who had a huge desmoid tumor on her left neck was treated by partial excision and postoperative irradiation. Detailed summary of this rare tumor are included with a review of the literature about treatment modality including irradiation. We suggest that local irradiation is one of the effective treatment tools in the management of desmoid tumor, especially, in the case of unresectable or postoperative residual tumors on the neck.