Background. The clinical relevance of Aspergillus fumigatus (Af) in cystic fibrosis (CF) is controversial. The aims of the study were to assess the prevalence of Af disease in our cohort of CF patients and evaluate whether allergic bronchopulmonary aspergillosis (ABPA) and sensitization to Af affected lung function, body mass index (BMI) and exacerbations. Methods. Clinical data and lung function of CF patients aged 6–18 years followed at the CF Centre of Parma (Italy) were recorded. Patients were classified as: patients with no signs of Af, patients sensitized or colonized by Af, patients with ABPA or patients with Aspergillus bronchitis (Ab). Results. Of 38 CF patients (14.2 yrs (6.2–18.8) M 23), 8 (21%) showed Af sensitization, 7 (18.4%) showed ABPA, 1 (2.6%) showed Af colonization and 1 (2.6%) showed Ab. Compared to non-ABPA, patients with ABPA had lower BMI (15.9 ± 1.6 vs. 19.7 ± 3.4, p < 0.005), lower lung function (FEV1 61.5 ± 25.9% vs. 92.3 ± 19.3%, p < 0.001) and more exacerbations/year (4.43 ± 2.44 vs. 1.74 ± 2.33, p < 0.005). Patients with Af sensitization showed more exacerbations/year than non-Af patients (3.5 ± 3.2 vs. 0.9 ± 1.2, p < 0.005). ABPA and sensitized patients had more abnormalities on chest CT scans. Conclusion. This study showed the relevant clinical impact of ABPA and Af sensitization in terms of exacerbations and lung structural damage.