SUMMARY A 35 week pre-term male neonate with a prenatal history of polyhydroamnios and intrauterine growth retardation was delivered from a 25 year old mother. Chest radiograph and CT showed pleural effusion, and mediastinal shift to the right. The patient undervent an exploratory laparotomy and a posterolateral left diaphragmatic hernia was identified as well as 3mm perforation of a normally positioned cecum. The left pleural space was cleared of debris, then the diaphragmatic defect was closed. After appendectomy and colonic biopsy cecostomy was performed. His postoperative course was uneventfull. However, the infant presented a spastic posture, opistotonic movement and tonic-clonic seizures. Cystic encephalomalasic areas in both hemispheres, loss of volume in white matter and cortical atrophy were detected by MRI. Performing colostomy closure he was discharged to the pediatric neurology department. At 8 months and 3 years follow up, the child has no problems due to CDH, but he has very severe neurologic deficit and is mentally retarded.