Abstract
Nerve sheath myxomas are rare, benign neoplasms of nerve sheath origin. They are often found arising from the dermis and subcutaneous tissues at the head and neck region. However, presentation in the oral cavity is rather unusual.
We report a case of a 33-year-old woman who presented with a 12-month history of an extensively large, firm swelling in the left buccal space extending to the retromolar area, resulting in a slight facial asymmetry. A well-defined radiopaque mass was seen on magnetic resonance imaging studies. The diagnosis of oral nerve sheath myxoma was established, adding to the literature its incidence and subsequently improving understanding of its clinicopathologic features, histogenesis, and distinct entity relative to other oral lesions that can mimic its presentation.