A wide spectrum of respiratory tract involvement may be seen in Sjögren's syndrome, however, multiple manifestations in one patient are unusual. A 65-year-old woman with a 5-year history of Sjögren's syndrome presented with hoarseness and pulmonary infiltrates. Lymphocytic interstitial pneumonitis, tracheobronchitis with subglottic edema and obstructive small airways disease were initially documented. Dramatic radiographic improvement of the lymphocytic interstitial pneumonitis occurred during 14 months of corticosteroid therapy. However, a rare primary malignant lymphoma of the trachea developed and presented with steroid refractory, upper airways obstruction. Subsequent patient management and the dichotomous behavior of the lymphoid infiltrates are discussed.