Mycobacterium avium-intracellulare complex (MAC) is one of the most common forms of non-tuberculous mycobacterial (NTM) infection. MAC is a ubiquitous bacterium that resides in both natural and man-made environments. Surgical intervention is well established in NTM infections causing cervical lymphadenitis, but its role in airway disease is not well understood.Invasive pulmonary infection is usually associated with immunocompromised patients, but it occurs in otherwise healthy children as well.We present a challenging clinical case of an 18-month-old female with severe mediastinal MAC causing bilateral bronchogenic obstruction and respiratory compromise requiring emergent intubation and intervention, likely due to a genetic predisposition secondary to Interferon Gamma Receptor 2 (IFNGR2) haploinsufficiency.During the initial bronchoscopy, the left bronchus was 99% obstructed while the right bronchus was 60% obstructed. The right lesion was biopsied and drained whitish fluid with improvement in clinical status shortly thereafter. A culture was sent. Follow-up bronchoscopy with excision of residual right mass allowed for extubation in the operating room with discharge on azithromycin, rifabutin, and ethambutol. Repeat bronchoscopy after discharge revealed recurrence of bilateral lesions. The patient was started on nebulized amikacin in addition to her current regimen with full resolution after treatment. Despite subtotal removal of MAC lesions possibly increasing the chances of recurrence, surgical intervention in this patient resulted in rapid improvement in respiratory status, and it may represent the preferred treatment in patients with any airway concerns.