Although congenital defects of diaphragma often occur in the period immediately following birth, 10-20% of these cases are diagnosed later. We report on a 7-month-old male infant with late-presenting congenital diaphragmatic hernia associated with a thoracic ectopic kidney. We conclude that congenital diaphragmatic defects should be considered in young children with respiratory distress and that computerized tomography is a noninvasive and accurate diagnostic method in the evaluation of additional abnormalities in these patients. © Springer-Verlag 2006.