A 55-year-old man was admitted to our hospital because of progressive dyspnea. Chest radiography showed interstitial shadows in the upper lobe. The pathological diagnosis of lung biopsy specimens obtained from upper and middle lobes by video-assisted thoracoscopy was non-specific interstitial pneumonia, group 2. Administration of glucocorticoids improved the symptoms, the chest radiography findings, and the serum KL-6 level. This patient may belong to the new category of idiopathic interstitial pneumonia, though he exhibited several features of idiopathic pulmonary upper lobe fibrosis originally described by Amitani et al.