Objectives: 1] To describe a case of an embryonal rhabdomyosarcoma presenting as al radiolucent mandibular mass in a 3-year-old child. 2] To review existing literature on the clinical picture and pathophysiology of intraosseous rhabdomyosarcoma. 3] To identify learning points in the diagnosis of intraosseous rhabdomyosarcoma. Design: Case report. Setting: A tertiary referral hospital. Patients: One (1) Results: A case of a 3-year-old child with a radiolucent mandibular mass is described. Thel final histopathologic report turned out to be embryonal rhabdomyosarcoma. Intraosseous rhabdomyosarcomas are rare occurrences (3.5 percent in one review), and clinically present in younger age groups, with a non-tender, enlarging, firm-hard mass over a specific area. A review of the available literature on intraosseous rhabdomyosarcomas, and its proposed pathogenesis, is presented. Conclusion: A case of a radiolucent mandibular mass in a 3-year-old child is presented. Intraosseous rhabdomyosarcomas of the mandible are rare occurrences that pose challenges to the otorhinolaryngologist. Taken separately, the presentation, patient characteristics, clinical course, ancillary laboratories and imaging modalities may lead even the most astute otorhinolaryngologist astray. The whole clinical picture should be taken together so that the correct diagnosis will not be missed despite the rare presentation. (Author)