Background and Aims Pulmonary Mucoepidermoid carcinoma (MEC) accounts for 0.1-0.2% of all lung cancer. It occurred in the 3-78 years old, and 50% patients younger than 30. MEC has no standard treatment, but recently reports indicated MEC without epidermal growth factor receptor (EGFR) mutations sensitive to gefitinib. Objectives To explore a new standard treatment strategy for MEC, after reviewed literature related to MEC, we used Gefitinib to treatment a patient with EGFR-negative MEC, and observe its effects. Methods 10-year-old boy was diagnosed with low-grade MEC by bronchial lung biopsy, EGFR gene mutation test was negative. Gefitinib was administered as neoadjuvant therapy at 125 mg daily. Results The patient underwent right middle lobe, lower lobe resection and mediastinal lymph node dissection. After surgery, the patient had gained weight (5 kg) after 18 days of gefitinib therapy. A CT scan of the chest 1 month after surgical resection showed no recurrence, and followed for 22 months after treatment without tumour recurrence, suggesting that the patient was completely cured. Conclusion Gefitinib has potential to become a standard treatment for pulmonary MEC patients, including pediatric patients. However, the mechanisms need further investigation. [ABSTRACT FROM AUTHOR]