A case of mandibular myofibroma in a 2-month-old boy is presented. Including this case, 24 pediatric and 11 adult patients with maxillofacial myofibroma have been reported since 1981. Of the 24 pediatric patients, 15 (62.5%) had lesions affecting the mandible. The adult cases had no mandibular involvement. Histologic evaluation of the tissue specimen revealed an interlacing pattern of spindle-shaped cells with long oval nuclei. Tissue immunohistochemical staining found it to be reactive for antibodies directed against vimentin and alpha-smooth muscle actin, but not desmin, S-100 protein, neuron-specific enolase, or myoglobin. Electron microscopy examination revealed the following cells: myofibroblast-like cells, fibroblast-like cells, and intermediate cells that were similar to the fibroblast-like cells except for the presence of a few microfilaments. Myoblast-like cells were not seen.