Rhabdomyosarcoma is the most common soft-tissue sarcoma of the head and neck region in children and adolescents. Oral cavity involvement is relatively uncommon, with tongue, soft palate, hard palate, and buccal mucosa being the sites of predilection. This report presents a rare case of intraosseous oral rhabdomyosarcoma arising in the mandibular bone of a 6-year-old child. Clinical, radiologic, and histopathologic features and possible pathogenesis are discussed.
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