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Low-grade fibromyxoid sarcoma (LGFMS), a soft tissue tumor that has high recurrence and metastasizing potential, rarely occurs in the head and neck region. Therefore, the treatment for LGFMS in the facial area is challenging in terms of cosmetic and functional maintenance. The authors report a case of LGFMS in the posterior nasal cavity. It was completely removed in parallel with the nasal endoscopic and oral approach, but the lower margin was closed by preserving the soft palate. The patient is well without any recurrence or metastasis after 5 years of follow-up.