Extralobar sequestration of lung is a rare congenital malformation frequently diagnosed during repair of congenital diaphragmatic hernia. However, the combined association of congenital diaphragmatic hernia with both pulmonary sequestration and malrotation of gut is rare. We report a case of a 1-year-old girl with extralobar sequestration of lung and malrotation of gut detected during the repair of diaphragmatic hernia. The histopathological examination of the sequestered lobe revealed dilated bronchioles, alveolar ducts and alveoli along with dilated subpleural and peribronchiolar lymphatics and areas of type II congenital pulmonary airway malformation.