Chronic interstitial lung diseases in children represent a heterogeneous group of disorders of both known and unknown causes that share common histological features. Despite many efforts these diseases continue to present clinical management dilemmas, principally because of their rare frequency that limits considerably the possibilities of collecting enough cases for clinical and research studies. Through a Task Force conducted by the European Respiratory Society, which comprised respiratory physicians and basic scientists from across Europe, 185 cases of interstitial lung diseases in immunocompetent children were collected and reviewed. The present report provides important clinically-relevant information on the current approach to diagnosis and management of chronic interstitial lung diseases in children. In addition, recommendations for the management of paediatric interstitial lung diseases, as well as new insights into interstitial lung diseases pathophysiology during childhood are discussed.