Rhabdomyosarcoma (RMS) of the parotid gland is an exceedingly rare malignancy in children, accounting for less than 1.5% of salivary gland tumors, with a high-grade aggressive nature and poor prognosis. We report a case of a 15-year-old female presenting with a painless, progressively enlarging swelling in the right preauricular region with associated facial nerve paresis and systemic nodules. Diagnostic imaging revealed extensive lesion involvement in the parotid, masticator, and retromaxillary spaces with distant metastases. Histopathology and immunohistochemistry confirmed embryonalrhabdomyosarcoma. Despite initial response to chemotherapy with the VAC regimen, the tumor recurred within nine months, necessitating orbital exenteration for metastatic orbital involvement. This case highlights the importance of early multidisciplinary management combining surgery, radiation, and chemotherapy for improving outcomes in this rare and aggressive malignancy. Inclusion of such cases in multicenter trials is essential to advance therapeutic strategies.

Rhabdomyosarcoma (RMS) is a high-grade malignant neoplasm arising from mesenchymal cells, typically seen in the pediatric population, and is rarely encountered in adults. Embryonal rhabdomyosarcoma (ERMS) of the nasal cavity in adults is particularly uncommon and poses diagnostic challenges due to its non-specific presentation and aggressive behavior. We report a rare case of a 19-year-old male presenting with nasal obstruction, proptosis, and noisy breathing. Imaging revealed a large, invasive soft tissue mass involving the nasal cavity, orbit, and intracranial structures. Histopathology and immunohistochemistry confirmed the diagnosis of ERMS. The patient was managed with chemoradiotherapy (VAC regimen with 66Gy RT). This case emphasizes the need for high suspicion and prompt intervention for better outcomes in such rare adult presentations of ERMS.