Intraosseous mucoepidermoid carcinoma (IMC) is a rare malignant tumor of the jawbones and a rare subtype of salivary gland neoplasms. The pathogenesis of IMC remains unclear. Clinically, it needs to be differentiated from other jaw lesions such as odontogenic jaw cysts, keratocystic odontogenic tumors, ameloblastomas, and central squamous cell carcinomas of the jaws. Radical surgery is considered the optimal treatment. This article reports a case of well-differentiated IMC of the mandible and discusses its clinical features, diagnosis, and treatment options based on relevant literature.