ObjectiveTo summarize the clinical features of pulmonary mucoepidermoid carcinoma (PMEC) and improve the level of the diagnosis and therapy.MethodsA case of PMEC was reported and related literatures were reviewed in PubMed, WanFang data and China National Knowledge Infrastructure.ResultsA 21-year-old female patient, complaining of cough and expectoration for 5 months, intermittent hemoptysis for 3 months and repeated fever more than 4 days, was hospitalized in March 2017. After admission, the patient was diagnosed as low-grade PMEC and received anti-infection, fibrobronchoscope sputum aspiration and alveolar lavage therapy. During the hospitalization, the anti-infection of the patients was not much effective, and the chest CT examination suggested space-occupying lesions in the middle lobe of the right lung. After the surgical removal of the right lung, the syndrome of the patient was improved. A total of 13 patients were reviewed, there was no obvious gender difference, and the median age was about 40 years old (most frequently occurred in younger adults with an average age less than 50 years). Frequent clinical manifestations were cough, fever and hemoptysis, which were often accompanied with atelectasis. Most of the patients could be improved after surgical resection, and a few need comprehensive treatment including radiotherapy, chemotherapy and targeted treatment, etc.ConclusionsPMEC is rare and the etiology is still unclear. The pathology is mainly low-grade. Majority of patients with PMEC could be cured after surgical resection.