Primary pulmonary mucoepidermoid carcinoma (PMEC) is extremely rare. Herein, we report a case of a 71‐year‐old male patient with high‐grade PMEC involving the right upper lobe that was successfully resected via lobectomy. As a result of invasion into the pleural and paratracheal lymph nodes, four cycles of adjuvant chemotherapy with paclitaxel and carboplatin were administered. There were no signs of relapse during 10 months of follow‐up. Furthermore, we reviewed the literature and summarized the surgical approaches, prognostic factors, and underlying genetic mechanisms of PMEC, which will benefit clinical treatment.