We report a rare case of isolated neurofibroma arising in the left mandible without family history of neurofibromatosis type 1. The tumor was histopathologically analyzed and an immunohistochemical panel comprising S-100 protein was performed. Vim, HMB45, and HHF35 were negative. The tumor cells were fusiform in shape, arranged in a plexiform manner, and grew actively. Features typical of neurofibroma, including an enlarged nerve fascicle composed of elongated nuclei and scant cytoplasmic cells, were identified. Our study suggest that neurofibroma can occur in the jaw as an isolated benign tumor in patients without other features of neurofibromatosis type 1.