Interstitial lung disease in children is a complex group of disorders whose etiology and pathogenesis is not entirely clear. Although the basic pathogenesis has been extrapolated from adult studies, its relevance in the pediatric population can be questioned. Several classifications of this condition have been put forward but Liebow's histologic classification is well accepted. Clinical manifestations are non-specific and can vary from no symptoms and a positive chest X-ray to more characteristic signs and symptoms. The triad of tachypnea, intercostal retractions and dry crackles is characteristic. Chest roentgenograms are a useful diagnostic tool and CT scans help to stage disease severity. Spirometry classically shows a restrictive pattern. Lung biopsy is the gold standard for establishing a diagnosis. Corticosteroids are the cornerstone of treatment in this condition. Thus, in view of the multifactorial nature of this disease, more extensive multi-centre prospective studies are required in order to better understand this rather complex group of disorders.