Isolated endobronchial lesions caused by Mycobacterium avium are rare, especially in the pediatric population. We share the case of a 10-month-old boy who, after 1 week of cough and low-grade fever, had a radiographic examination showing endobronchial obstruction. At bronchoscopy, a granuloma of the left bronchus intermedius was found. Histopathologic examination revealed necrotizing granulomatous inflammation. Kinyoun Acid Fast stain revealed acid fast bacilli. Cultures were positive for M. avium. Current treatment options and controversies are presented. The roles of antibiotics and steroids in preventing progressive disease are discussed. The need for serial bronchoscopy and the potential benefits of surgical resection are discussed. Isolated endobronchial M. avium infection remains a rare and challenging problem. The paucity of clinical experience, and variation in patient presentation, obligates a high index of suspicion, and frequent follow-up with bronchoscopic examination and pulmonary assessment, for the child diagnosed with isolated endobronchial atypical mycobacterial infection.