Myxofibrosarcoma, also known as a myxoid variant of a malignant fibrous histiocytoma (MFH), is one of the most common sarcomas in the extremities of elderly people. The lesion is characterized by a high frequency of local recurrence but is uncommon in the head and neck regions. Low-grade myxofibrosarcoma, which is commonly misinterpreted as being benign, has a tendency for histological and biological progression in local recurrences, highlighting the importance of an accurate diagnosis and wide surgical excision of the primary lesion. We report a rare case of low-grade myxofibrosarcoma of the mandible located in the left mandibular body and angle area. The tumor was first diagnosed as a myxofibroma and was resected initially. After the final biopsy the patient underwent combined chemo-radiotherapy. The progress of the patent was uneventful until the one year follow up.