<b>Introduction:</b> Desmoid tumors are rare and locally aggressive neoplasms originating from fibroblasts, characterized by a variable and often unpredictable clinical course.<b>Case report:</b> We present the case of a 43-year-old man with a neck lump who was diagnosed with a desmoid tumor of the left periverterbal space adjacent to the C5 and C6 roots of the brachial plexus.<b>Conclusions:</b> Treatment of desmoid tumors is challenging. Each case should be approached individually, and the course of action depends on the location and symptomatology of the tumor. Available management options include: active surveillance, systemic treatment (chemotherapy, hormone therapy and molecular drugs), radiotherapy and surgery.