We treated a patient with Sjögren's syndrome associated with multiple bullae and pulmonary nodular amyloidosis, both of which were identified by open lung biopsy. The mechanism of bullae formation appeared to be narrowing of the airway, as a result of extensive inflammatory cell infiltration to the bronchiolar wall, which acted as the check valve mechanism. We believe this to be the first reported case of Sjögren's syndrome accompanied by these two pulmonary manifestations.