Mucoepidermoid carcinoma of the lung presenting in childhood is an uncommon neoplasm. Symptoms of progressive bronchial obstruction proceed the recognition of this lesion. The well-confined local growth and minimal metastatic potential of childhood mucoepidermoid carcinoma make local resection with maximum conservation of pulmonary parenchyma the recommended operative treatment. Adequate surgical resection precludes the need for further radiation therapy or chemotherapy. This report details the presentation and treatment of a child with this rare tumor and reviews the present literature experience with childhood mucoepidermoid carcinoma.