Introduction: Low grade fibromyxoid sarcoma is a distinctive variant of fibrosarcoma. We recently experienced a rare case of low grade fibromyxoid sarcoma arising in the masseter muscle.
Case presentation: A 63-year-old Korean woman with a large growing mass in her right parotid gland area for 1 year visited our clinic. Complete removal of the tumor was achieved by parotidectomy with facial nerve preservation. The tumor measured over 4cm with pathologic findings compatible with low grade fibromyxoid sarcoma.
Conclusions: Low grade fibromyxoid sarcoma is an extremely rare tumor, and report of the present case is noteworthy since it represents a rare localization of low grade fibromyxoid sarcoma in the head and neck. Close follow up on a long-term basis is considered necessary because of its high potential to metastasize.