Introduction: Rhabdomyosarcoma (RMS) is a malignant mesenchymal neoplasm. It is one of the most common malignant head and neck tumors in children and the most frequent soft-tissue sarcoma. The main locations are the base of the skull and the nasopharynx. We present a rare case of a child presenting with parotid RMS. Presentation of Case: A 4-year-old male was admitted to our hospital with the chief complaints of a progressively enlarging, cervicofacial swelling. There was no history of fever or any other constitutional symptoms. Physical examination revealed a 60 mm left cervicofacial swelling, trismus and grade IV left peripheral facial paralysis. There were no fever or palpable nodes. Biological findings showed an increased LDH. Computed tomography scan of the neck revealed a bulky tumor in the left cervicofacial region, involving both superficial and deep lobes of parotid gland, causing compression of oropharynx, extending to the left cervical lymph nodes measuring 77*55 mm with difficulties determining the starting point. Two days after his admission, he presented dyspnea and fever. A tracheotomy was performed associated to cervical biopsy under general anesthesia. The immunohistochemical analysis revealed intense positivity for desmin and myogenin favoring the diagnosis of embryonal rhabdomyosarcoma. The patient received chemotherapy and radiotherapy. A residual mass remained in the parotid region, in contact with the carotid artery. Tumor resection was discussed but ruled out due to high surgical risk. Conclusion: Parotid RMS is a rare entity. Treatment follows a rigorous international protocol associating surgery, chemotherapy and radiation therapy.