The records of 73 patients with diaphragmatic hernia (CDH) with late manifestation which admitted to three different centers (Amirkabir and Children's hospitals two main referral centers for pediatric surgery (in Tehran and Tabriz respectively, and a private hospital) were reviewed. Clinical and radiological findings and operative data of 25 patients with CDH with delayed onset were analyzed. Age range was 2 months to 6 years, 15 males and 10 girls, 23 had left sided hernia and 2 had right sided. Presenting features included: chronic respiratory symptoms in 25 patients (60%), gastrointestinal (GI) complaints in 8 patients (32%), and respiratory distress in one patient (4%).The defect was an incidental finding in one patient (4%). One patient had previously normal chest radiograph. 10 patients (40%) were originally misdiagnosed clinically and radiologically as having either infective lung changes, or pneumothorax Inappropriate thoracocentesis had been performed in one case, misdiagnosed as having a pneumothorax. Operative repair was performed for all patients with abdominal approach. At operation 4 peritoneal sacs (16%) were found and all were excised. Intestinal malrotaion was most common anomaly (n=15 60%).None had evidence of pulmonary hypoplasia. One death occurred as a result of inappropriate thoracocentesis. Congenital diaphragmatic hernia did present beyond the neonatal period in significant number in this series (34.2%). Therefore the symptoms, signs and radiologic findings of patients with CDH presenting after the neonatal period may be difficult to interpret and may result in diagnostic delay, misguided therapy, and a potentially fatal outcome.It is the purposes of this study to familiarize the physicians and podiatrists with presenting features, diagnostic difficulties and errors, differential diagnosis and complications in late presenting CDH. In the hope that this will facilitate the diagnosis and management of this potentially dangerous disease to prevent mortality and morbidity. The interval between diagnosis and surgical therapy should be as short as possible. Early diagnosis is important as early surgery is recommended even in those without symptoms.