Embryonal rhabdomyosarcoma (RMS) of the nasosinusal region is an uncommon pediatric malignancy, with an estimated incidence of 4.3 cases per million children annually. This report presents a case of embryonal nasosinusal RMS in a 12-year-old child managed at our institution, alongside a review of the literature. We discuss the epidemiology, diagnostic challenges, and therapeutic approaches associated with this rare tumor, providing insights to enhance clinical management and improve prognosis in pediatric patients.