Desmoid tumors (fibromatosis) are rare benign tumors, they arise from musculoaponeurotic structures throughout the body. They are locally infiltrative, resulting in a high rate of local recurrence following surgical resection. Due to the rarity of these tumors in the head and neck region, we report a case of a patient with a desmoid tumor in the upper neck that was diagnosed and treated in our institution, to increase the awareness of the Otolaryngology-Head and Neck surgeons, and report available treatment options of this condition.