Fibromyxoma is a rare mesenchymal tumor that is benign, but locally invasive. It is a slow-glowing painless tumor with the potential for extensile bony destruction and cortical expansion and shows a relatively high recurrence rate. Fibromyxoma is found predominantly in the jaw, with the mandible more frequently affected than the maxilla. We recently experienced a case of fibromyxoma originating from the maxilla in a 50-year-old woman who complained of swelling on the right side of her cheek. En bloc resection via a sublabial approach and middle meatal antrostomy were performed. A diagnosis of fibromyxoma was based on pathologic findings. No recurrence or locally residual lesion has been found during 2-years follow up. Therefore, we present this rare case with a review of the literature.