Abstract: Objective　To explore the clinical manifestations and radiological features of cystic fibrosis (CF) in children. Methods　The clinical and radiographic data of 5 CF patients were retrospectively analyzed. Results　Among the 5 cases, there are 3 males and 2 females, aging from 2 to 13 years old (median age 6). Four of the five cases had complaints of repeated productive cough with or without fever and short breath. Pseudomonas aeruginosa was positive in sputum culture of three cases. Chest CT showed pneumonia and bronchiectasis with peribronchial thickening and mucus plugging. Paranasal CT showed frontal sinus agenesis and sinusitis with sticky secretion. The other one of the 5 cases had a complaint of abnormal hepatic function. The abdominal MRI showed liver cirrhosis and high signal intensity in the periportal area on T1-weighted imaging. Chest CT showed air trapping from small airways obstruction and bronchiectasis with sputum plugging. Five recurrent and two novel CFTR mutations were identified in all of the 5 cases. Conclusions　The radiographic findings of CF are characteristic, and of great significance to the clinical diagnosis of CF. The gene mutations of CF in Chinese are different from those in Caucasians.