Abstract： Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by the functional defect of cystic fibrosis transmembrane conductance regulator (CFTR). Allergic bronchopulmonary aspergillosis (ABPA) is one of the most serious aspergillus fumigatus related diseases, which mostly occurs in patients with CF and asthma.CF complicated with ABPA can affect multiple systems throughout the body.The clinical symptoms are complex and diverse, mainly related to respiratory symptoms, including cough, expectoration, wheezing, hemoptysis, and so on.Imaging manifestations include pulmonary inflammatory infiltration and central (proximal) bronchiectasis.Other system manifestations involve pancreatitis, steatorrhea, hepatosplenomegaly, etc.This article will summarize the diagnosis and treatment of a CF patient with ABPA whose first symptoms were repeated cough, expectoration, and aggravated hemoptysis.The relevant literature will also be reviewed, so as to raise clinicians′ attention to CF patients with ABPA and to improve their sensitivity to such diseases.