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Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents. Most patients present with a mass in the head and neck region, urogenital region, or with distal extremity involvement. The authors describe a challenging case of a 6-year-old male patient presenting with mandibular RMS. The clinical/radiographic/tomographic evaluations classified the tumor as an advanced stage (stage IV), with a mass of 6.0 cm involving the left side of the mandible and parotid region. The biopsy revealed round, spindled, and pleomorphic cells with hyperchromatic nuclei and rare larger rhabdomyoblasts with eosinophilic cytoplasm. The diagnosis was of embryonal RMS. The patient was referred for treatment with cycles of chemotherapy; however, pulmonary and bone marrow metastasis were identified. Radiotherapy and local surgery with microvascular reconstruction were performed later; however, the patient died after a few months. Early diagnosis is critical for a good prognosis and cure of patients with RMS. Correct diagnosis considering also the histological subtype is important for adequate treatment, which according to the literature is not uniform probably because of the rarity of this neoplasm.