Ljmphocytic interstitial pneumonia (LIP) is a rare form of interstitial pneumonia. It can occur as a idiopathic disease however most frequently associated with other diseases, particularly Sjogren syndrome. We present a 58 years old woman, with primary Sjögren syndrome who developed after 7 years interstitial lung disease. LIP was diagnosed on the basis of histological examinations of specimens obtained by open lung biopsy. Diffuse infiltrations composed of small polyclonal lymphocytes, plasma cells with reactive follicules were found. Infiltrates observed in bronchial and bronchiolar walls expanded into interlobular and alveolar septae. Prednisone in a dose of 40 mg per day was introduced and clinical, radiological, spirometric and gasometric improvement was observed. The dose of prednisone was gradually reduced but is still continued.