In this report, a four days old lamb was referred to Erciyes University, Pathology Department of Veterinary Medicine with a request of necropsy and reported with died from respiratory distress. At necropsy, it was seen that a portion of the stomach pass into the chest cavity from the 13 cm in length slit of diaphragm and found to compressing the left lobe of the lung. When the herniation brought to the normal position, the side in cleft part of diaphragm muscle was noticed that it was thinner. The left lobe of the lung was collapsed and also the presence of 8-12 cm sizes gray white discoloration was observed on liver. Microscopically, the rupture edges of the diaphragm muscle were composed of fibrous tissue. Atelectasis areas were seen in the lungs due from the stomach pressure and also fatty degeneration of the hepatocytes was observed in liver. This report was defined with results of necropsy and histopathological findings as a congenital diaphragmatic hernia which was the first case report in Turkey because of its rarity in lambs.