Objective: To integrate the available data published on central mucoepidermoid carcinoma (CMC) into a comprehensive analysis of its clinical aspects, histology, treatment, and prognostic factors. Methods: An electronic search was undertaken in July 2017. Eligibility criteria for publications included having clinical, histological, treatment, and time of follow-up data to confirm the diagnosis. Results: 36 publications (147 CMCs) were included. The lesion showed a slightly higher prevalence among women than men. There was a high prevalence in the fifth to seventh decades of life. The most common symptom was swelling and the most commonly observed area was the mandible. Histologically, low-grade tumors were the most common. The treatment of choice was tumor resection. In addition, gender, histological grade, conservative treatment, and lymph node metastasis were significant independent prognostic factors. Conclusion: Male patients with histological grade III CMC of the jaw bone, who used conservative treatment as the main treatment, and presented with lymph node metastasis, were more likely to have a worse prognosis. (c) 2017 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.