Childhood interstitial lung disease represents a rare and heterogeneous group of diseases that can result in significant morbidity and mortality, some leading to death during infancy. CT is the imaging test of choice. Although many CT findings are nonspecific and a definitive diagnosis usually cannot be reached by CT alone, the interpreting radiologist is instrumental in defining disease extent and refining the diagnosis. Chest CTs are of key importance in guiding site selection for lung biopsy and for following disease progression and response to treatment. Thus, from the radiologist’s perspective, ensuring maximal quality of CT imaging and interpretation is paramount.