In this study we retrospectively analyzed the clinical characteristics of chronic hypersensitivity pneumonitis (CHP) with emphysema. Patients with CHP (n=161) diagnosed at our hospital were classified into two groups: CHP with emphysema [CHP-E(+) group] and CHP without emphysema [CHP-E(-) group]. The CHP-E(+) group consisted of 53 cases (33%) and had a higher frequency of men (92%) and a higher frequency of smokers (96%). The %DLco (54±25%) and %DLco/VA (56±22%) in pulmonary function tests were low, while %FVC (82±18%) and FEV1/FVC (78±9%) were normal. There were no differences between groups in the incidences of pulmonary hypertension or acute exacerbation of interstitial pneumonia, and the incidence of lung cancer was significantly higher in the CHP-E(+) group than in the CHP-E(-) group (24.5% vs. 4.6%, p<0.0001). The CHP-E(+) group manifested features observed earlier in patients with combined pulmonary fibrosis and emphysema.