This study provides a comprehensive analysis of 61 patients with combined pulmonary fibrosis and emphysema (CPFE), a condition that has not been comprehensively described. The patients, all smokers, presented with dyspnea on exertion, basal crackles, and finger clubbing. Pulmonary function tests revealed subnormal spirometry, severe impairment of gas exchange, and high prevalence of pulmonary hypertension. The presence of pulmonary arterial hypertension (PAH) at diagnosis was a critical determinant of prognosis, with a median survival of 6.1 years. The study highlights the distinct clinical, radiological, and functional characteristics of CPFE, emphasizing the need for accurate diagnosis and management.This study provides a comprehensive analysis of 61 patients with combined pulmonary fibrosis and emphysema (CPFE), a condition that has not been comprehensively described. The patients, all smokers, presented with dyspnea on exertion, basal crackles, and finger clubbing. Pulmonary function tests revealed subnormal spirometry, severe impairment of gas exchange, and high prevalence of pulmonary hypertension. The presence of pulmonary arterial hypertension (PAH) at diagnosis was a critical determinant of prognosis, with a median survival of 6.1 years. The study highlights the distinct clinical, radiological, and functional characteristics of CPFE, emphasizing the need for accurate diagnosis and management.