Background: Ossifying fibromyxoid tumor (OFMT) is defined as a soft tissue neoplasm with indefinite histological origin. Here we describe a case of malignant OFMT located in the infratemporal fossa, surgical approach, and to review the available literature describing this tumor. Methods: Case report and literature review. Results: We present the case of a 41-year-old man who was referred to our clinic with right preauricular swelling, right sided hearing loss, tinnitus, and feeling of ear fullness which was noted 8 months ago Initial radiological investigations showed right sided infratemporal fossa mass. Computed tomography-guided core needle biopsy was performed and the histopathological specimen showed spindle cell neoplasm with minimal nuclear pleomorphism. Tumor was excised through the transmandibular approach after which adjuvant radiation therapy was administered. One year after surgical treatment, the patient presented with right side Level II cervical neck mass indicating post-biopsy metastatic OFMT. The patient underwent right modified radical neck dissection for tumor removal without adjuvant chemoradiation. Conclusions: The reported case demonstrates that OFMT, although a rare tumor, can metastasize to adjacent lymph nodes. From our experience, we suggest performing cervical neck dissection once OFMT, especially the malignant type, is identified in the head and neck region.