Pulmonary mucoepidermoid carcinoma (PMEC) is an extremely rare tumor of the respiratory system. The clinical presentation of PMEC is variable and nonspecific, including cough, hemoptysis, and wheezing, and may mimic other symptoms of pneumonia or asthma. Here, we present a case of PMEC in a 12-year-old male who was diagnosed with and treated for asthma for 2 years. The patient presented with symptoms of respiratory failure that did not respond to steroids or bronchodilator medications. Chest computed tomography (CT) scans revealed an endotracheal tumor. The patient underwent complete tumor resection, with no signs of recurrence 6 months after treatment.