Background: Cystic fibrosis is a chronic inheritable disease presenting in newborns with lifelong complications. It is a common disease worldwide and in developing countries, like Saudi Arabia, a need for proper management is essential for the survival of affected children. Objective: The aim of this review was to evaluate the diagnostic and management approach of cystic fibrosis in the pediatric age group. Methods: We searched PubMed for (("Cystic Fibrosis"[MeSH]) AND (“Evaluation"[MeSH] OR "Management"[MeSH] OR "Diagnosis"[MeSH])) Conclusion: Complications and their prevention should be dutifully explained to parents as it would build their confidence as caretakers. Emphasis on diet and adherence to management plan would support the patient’s lifestyle. Combined treatment of Ivacaftor and lumacaftor helps reduce respiratory complications, hospitalizations, and antibiotic use in CF patients. Correct management of this illness is crucial to the child’s survival and multifactorial, with many afflicted reaching adulthood. Fortunately, there has been an improvement in the management of CF due to CFTR modulators, but most of them are for children older than 12 or 6-12 years of age and have side effects and high costs. The new era of research in this disease focuses on new and better drugs.