Occurrence of central mucoepidermoid carcinoma within the jaw bones from aberrant salivary tissues is extremely rare and accounts for 2%-4% of all central mucoepidermoid carcinoma. Mandible is more commonly affected than maxilla in a ratio of 2:1. The origin of mucoepidermoid carcinoma is controversial and questionable. Central mucoepidermoid carcinoma is frequently misdiagnosed radiographically and clinically as a benign odontogenic tumor or cyst. Hence, it is important to detect malignant signs and histopathological feature of central mucoepidermoid carcinoma of the mandible to confirm the diagnosis. This paper reports a case of central mucoepidermoid carcinoma of the mandible in a 56-year-old patient with review of literature.