Endobronchial mucoepidermoid tumors are rare neoplasms but most cases are reported in children. Due to nonspecific symptoms, diagnosis can be challenging, but early diagnosis and treatment are crucial for prognosis. We present the case of a boy, with chronic respiratory insufficiency due to bronchiolitis obliterans, that presented worsening exertional dyspnea at 12 years. Spirometry showed a frank deterioration of respiratory function and CT scan revealed an obstructive polypoid mass in the intermediate bronchus. Given the severe basal ventilatory compromise and risk associated with surgical treatment, rigid bronchoscopy and laser excision, and photocoagulation were performed, with clinical and functional improvement. The histological examination revealed a low-grade mucoepidermoid carcinoma. The option for a minimally invasive procedure requires careful follow-up due to the risk of tumor recurrence.