Late presentation of congenital diaphragmatic hernia is 5-30% of all congenital diaphragma hernia cases. It can present as Morgagni, Bochdalek and paraesophageal hernia. Misdiagnosis can result in significant morbiditiy. A 17-month-old girl presented with vomiting and abdominal pain. On physical examination, circulatory disturbance, cyanosis, abdominal distantion were present. Her O2 saturation was 60% and she was tachycardic (180 bpm) and tachypneic (58 bpm) with hypotension (60/35 mmhg). Patient's heart and mediastinum were shifted into the right hemithorax on the chest X-ray. Bowel loops in the left hemithorax with air-fluid levels were seen in her plain X-ray and diaphragmatic hernia was seen in her computed tomography examination. She was referred to our center and operated within an hour. Herniated intestinal loops and stomach were observed from about 2 cm diameter defect of diaphragma were repaired primarily. She was extubated in postoperative 4th day. Late presentation of congenital diaphragmatic hernia may be confused with many situation and is difficult to diagnose without clinical suspicion. Accurate diagnosis and urgent treatment is lifesaving.