Background: Rhabdomyosarcoma (RMS) is a rare malignancy, accounting for 3% of all cancers in children, as the most common primary site for RMS is in the head and neck region. The most common  histology in children is the embryonal subtype. The diagnosis of embryonal rhabdomyosarcoma in the head and neck region is primarily based on histopathology, following biopsy of the tumor. Despite the improved outcome based on the EFS and OS using the three different treatment modalities-chemotherapy, radiotherapy and surgical treatment, the survival of patients depends on their IRS groups—pathological and surgical.

Case presentation: We present a clinical case of a nine-year-old male with initial complaints of painful mass in the region near the left parotid gland. Clinical and paraclinical investigations were conducted for diagnostic purposes. The postoperative histopathology report given at the centre of primary wide excision and cervical neck dissection was embryonal rhabdomyosarcoma. The patient then was treated with adjuvant chemotherapy. The strategy of concurrent chemo-radiotherapy was not implemented, and radiotherapy was delayed for 4 months due to the patient’s refusal of the treatment.

Results: The combination of surgery, chemotherapy and local control with radiotherapy achieved a good result in our patient.

Conclusions: Rhabdomyosarcoma (RMS) is a predominantly pediatric malignancy which can present throughout the body including the head and neck region. The use of radiation therapy is an important part of multimodality treatment for rhabdomyosarcoma

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