Interstitial lung disease (ILD) in children represents a heterogeneous group of rare lung disorders characterised by an inflammatory process of the alveolar wall and the pulmonary interstitium, which share common clinical, radiological and histological findings. Clinical presentation of paediatric ILD is non-specific and variable. Respiratory signs and symptoms include tachypnoea, rapid shallow breathing, retractions, cyanosis and crackles. Failure to thrive and respiratory distress are common in infants. Dyspnoea on exertion is a symptom encountered in older children. Non-respiratory symptoms can be present when ILD is part of a systemic disorder. Although non-specific and variable, the clinical presentation at the time of diagnosis has some prognostic value.