A patient with primary Sjögren's syndrome complicated with primary biliary cirrhosis and lymphocytic interstitial pneumonia is presented. She was 38 years old and was admitted to our hospital for generalized pruritus, xerostomia, xerophthalmia, fatigue, and reticulonodular changes on her chest X-ray. With the findings of ground-glass attenuation and centrilobular nodules in high resolution computerized tomography, the diagnosis of lymphocytic interstitial pneumonia was made. Oral methylprednisolone 1 mg/kg/day and ursodeoxycholic acid 15 mg/kg/day were started. At the 6th month follow-up, she had no complaints, and pulmonary function tests and high resolution computerized tomography were normal. This is the first case of Sjögren's syndrome, primary biliary cirrhosis and lymphocytic interstitial pneumonia in the English literature.