Objective: To report a case of Desmoid Tumor in the cervical region. Case Report: A case of a large mass in the cervical region of a patient with progressive growth, neck pain and some move restriction, without other comorbidities. Imaging, histopathologic and immunohistochemical study were needed for diagnosis, surgical treatment and postoperative care. Imaging showed a 12.6 x 5.7 x 5.8cm tumor, T1-weighted muscle isointense and T2-weighted hyperintense heterogeneous core, close to paravertebral muscles and pushing left sternocleidomastoid muscle laterally. The chosen treatment was complete tumor exeresis. Immunohistochemistry revealed myofibroblastic neoplasm compatible with desmoid fibromatosis with positivity for nuclear beta-catenin. Final Considerations: Large benign and infiltrative tumors need to be well conducted. It is essential that otolaryngologists are aware of the nature of these cases, differential diagnosis, treatment options and their possible complications, because eventually cases like this may appear requiring special care.