Late presentation of congenital diaphragmatic hernia is uncommon. It poses considerable diagnostic challenges when it strangulates. The authors report a case of a left posterolateral strangulated congenital diaphragmatic hernia in a 5-year-old child diagnosed at the stage of acute intestinal occlusion with intestinal necrosis and managed successfully. A strangulated congenital diaphragmatic hernia should be suspected in the case of an association of sudden-onset respiratory and digestive manifestations with no sign of trauma or specific pulmonary history. It then requires an antero posterior thoracic X-ray or, even better, a thoracic-abdominal scan to confirm the diagnosis.