OBJECTIVE: Due to the great advances recently achieved in the treatment of Cystic Fibrosis as well as to the fact that pediatricians need to have a better understanding of this disease, the authors propose an extensive review of the subject. METHODS: We selected the most outstanding publications on Cystic Fibrosis in the international literature of the recent years, with the purpose of being up-to-date and at the same time offering a practical synthesis for the readers. RESULTS: We elaborated an extensive review about Cystic Fibrosis covering the following topics: historical remarks, genetics, physiopathogenesis, microbiology of pulmonary infections, clinical manifestations, clinical and laboratorial criteria for diagnosis, differential diagnosis, treatment and prognosis.