Abstract
Rhabdomyosarcoma is a malignant neoplasm of mesenchymal cells, showing varying degrees of striated muscle cell differentiation. The most common sites of occurrence are the head and neck (40%), genitourinary tract (25%), and extremities (20%). Rhabdomyosarcoma is anatomically divided into two categories including parameningeal and nonparameningeal. It predominantly occurs in children while rarely found in adults, and involvement of the oral cavity accounts for only 10%-20% of all head and neck cases. The present case is of oral rhabdomyosarcoma of a 27-year-old woman, involving the mandibular region and demonstrates its clinical, radiological, histological, and immunohistochemical findings.