This is a review of 122 articles published until December 2003 that describe 349 children with late-presenting postero-lateral congenital diaphragmatic hernia (CDH). Data regarding pre-operative diagnostic work-up were adequately reported in 177 patients with left CDH and in 41 with a right-sided defect. Chest radiography was the only diagnostic study in 92 (51.9%) children from the former group and in 17 (43.9%) from the latter group. In other patients, diagnostic work-up encompassed various combinations of two or more imaging modalities. Apart from chest radiography, contrast study of the upper gastrointestinal tract was the most frequently performed imaging modality. In 88 (25.2%) children, initial radiographic features of CDH were misinterpreted. Pneumothorax and pleural effusion were the most common initial findings. Analysis of the hernial content in this group of patients has shown that herniation of the stomach, spleen or omentum should be regarded as risk factors for misdiagnosis of left CDH, whereas for right CDH, the risk factor is the presence of liver in the chest. Late-presenting CDH may pose a significant diagnostic problem because of the great variability of radiographic appearance. Chest radiography following passage of a nasogastric tube and contrast studies of the gastrointestinal tract seem to be the most useful investigations for the diagnosis of left CDH. For patients with right CDH, owing to the high probability of liver herniation, a chest radiograph with liver scintigraphy or CT seems to be the best diagnostic option.