We present a case of a desmoid tumor recurrence in a patient with a history of a resected desmoid tumor of the right neck area with free surgical margins six months earlier. The neoplasm was found to invade the parapharyngeal space, and wide excision was performed including most of the sternocleidomastoid muscle (SCM), the thrombosed internal jugular vein (IJV), and the infiltrated spinal accessory nerve (SAN). The histopathologic findings displayed free microscopic margins, with close margins at the site of the parapharyngeal space extension. After 3 months, there was no sign of tumor recurrence. After 6 months, local tumor recurrence was identified on clinical examination and imaging. The decision of the Oncology Board was further treatment with radiotherapy (RT). Response to treatment was satisfactory, and the patient was on close follow-up for twelve months. Desmoid tumors are very rare benign neoplasms of mesenchymal origin with negligible mortality but high morbidity, due to their high recurrence rates, local tissue infiltration, and unpredictable disease course and response to treatment. No universally acceptable treatment protocols have been introduced to date. Appropriate patient counseling and close follow-up are warranted in all cases.