Low-grade myofibroblastic sarcoma is a malignant tumor arising from myofibroblasts, which has only recently become clearly defined. It represents a rare entity developing in the soft tissues of the head and neck. About 20 cases have been reported in the oral cavity, especially in the tongue and bone, while gingiva as the primary site has been described only twice to date. Diagnostic methods include histology and immunohistochemistry. The present report concerns a case of a 13-year-old female child who presented with gingival nonulcerated swelling that was interpreted for a long time as a gingival fibroma. A low-grade myofibroblastic sarcoma was diagnosed and the patient underwent a segmental osteotomy of the right posterior region of the maxilla. There was no sign of recurrence or metastatic disease during the 12-month postoperative period.
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