Combined pulmonary fibrosis and emphysema syndrome (CPFE syndrome) is the co-existence of emphysema and pulmonary fibrosis in individuals. The syndrome was first described by Cottin et al., in 2005 and is characterised by upper lobe emphysema and lower lobe interstitial fibrosis. Patients with CPFE show severe dyspnoea and hypoxemia with exercise. Lung function tests (LFT) reveal a mixed pattern with relatively preserved lung volumes and severely low diffusing capacity for carbon monoxide (DLCO). CPFE is prevalent in heavy male smokers and have a high probability of developing pulmonary hypertension, acute lung injury and lung cancer