A 49-year-old man was referred to our hospital because of progressive volume reduction in bilateral upper lobes over 10 years. He was slim, with a straight back. Chest high-resolution computed tomography (HRCT) showed consolidation with small radiolucent areas in both upper lobes. Surgical biopsy by video-assisted thoracoscopy was performed in the right upper and lower lobes. The pathological findings of the upper lobe showed intraluminal fibrosis with alveolar collapse and proliferation of elastic fibers on the subpleural surface, but the lower lobe was normal. We diagnosed his illness as typical idiopathic pulmonary upper lobe fibrosis (IPUF), previously described by Amitani in 1992.