Low-grade fibromyxoid sarcoma (LGFMS) represents a rare, indolent, benign neoplasm that proliferates at various anatomical locations. Histologically, the tumor consists of bland, deceptively benign-appearing spindle cells in a whirling pattern, as well as myxoid and collagenized stroma. The current treatment includes surgical excision. The aim of this investigation was to analyze a rare clinical case of recurrent LGFMS of the neck. Relevant literature is briefly reviewed. An 81-year-old male was admitted with an enlarging painless nontender mass in the neck. The patient referred a past medical history of LGFMS. Magnetic Resonance Imaging (MRI) depicted a large, well-defined mass in the lower neck and supraclavicular area, extending till the left upper area of the anterior thoracic wall. Based on the imaging characteristics and the medical history, a wide surgical excision of a 5.6 × 6.6 × 3.3 cm area, in close proximity to the cervical vertebrae was performed. On microsopic examination, the tumor contained alternating fibrous and mixoid components, with mixed hypo- and hyper-cellular areas, minimal necrosis and negligible cellular pleomorphism or nuclear atypia. As LGFMS is a soft tissue malignancy, any recurrences are usually local; therefore treatment of choice is radical, wide margin surgical excision. Nevertheless, the ideal goal of large safety margin of healthy tissue might be unattainable if it requires resection of vital anatomic structures. Because LGFMS has a very low mitotic rate, neither chemotherapy nor radiotherapy is expected to have significant effect on long-term prognosis.