Cemento-ossifying fibroma is a benign neoplasm that arising from jaw bones. Here, we report a rare case of cemento-ossifying fibroma occurred at maxilla in a 18 year-old female who presented an asymptomatic bulging out mass at left maxilla. She underwent enucleation with curettage of the lesion under local anesthesia and there were no complications upon 2 weeks follow- up and no recurrence noted 1 year post-operatively. In reviewing articles, cemento-ossifying fibroma arise from the stem cells of periodontal ligament surrounding teeth. It is a well-demarcated lesion composed of fibrocellular tissue, lamellar bone, woven bone and acellular deposition. The clinical features, histopathological findings of this disease will be reviewed and the differences of the current case were compared. In general, it is a benign neoplasm but may recur in some cases.