Lymphomatoid granulomatosis (LG) is an extremely rare disease and is an unusual cause of central airway obstruction (CAO) with no standard of treatment in these conditions. LG is characterized by angioinvasion and angioinfiltration along with lymphohistiocytic cells. We present a 21-year-old female with LG who developed endobronchial lesions causing malignant CAO and acute hypoxic respiratory failure. She was treated with argon plasma coagulation, as well as a self-expandable metallic stent in the left main bronchus. Her stent was removed 4 months later after chemotherapy. Endobronchial stenting may be a useful bridge in patients who are undergoing more definitive treatment.