We present a rare case of intraoral nerve sheath myxoma. Clinically, the neoplasm mimics many other oral mucosal pathosis, underscoring the importance of histopathology in ensuring accurate diagnosis of oral mucosal lesions. Reports of intraoral nerve sheath myxomas are essential to enhance our understanding of this rare intraoral entity.
Keywords: S100; nerve sheath myxoma; neurothekeoma; oral pathology.