Here, we report a case of nodal marginal zone lymphoma in primary Sjogren's syndrome (SS) presenting with findings of pulmonary involvement. A 46-year-old woman was admitted to our hospital with fatigue, cough, dyspnea, xerostomia and weight loss. On physical examinations cervical and supraclavicular lymphadenopathies were noted. Chest X-P and computed tomographic films demonstrated reticular shadows in the bilateral lower lung and transbronchial lung biopsy revealed desquamative interstitial pneumonitis (DIP). Although immunoserological tests were negative, this patient was diagnosed as primary SS by sicca symptoms, positive Schirmer tear test and labial gland biopsy. Excisional biopsy of cervical lymph node revealed the presence of nodal marginal zone lymphoma
