We report a 13-year-old girl with an unusual, complex bronchopulmonary foregut malformation. The malformation included extralobar pulmonary sequestration and a duplication cyst of mixed bronchogenic and oesophageal type. Preoperative CT and MRI demonstrated the cystic and solid portions of the mass and indicated an aberrant vascular supply, suggesting the possibility of bronchopulmonary foregut malformation and several other differential diagnoses. A direct communication between the cyst and the bronchus of the sequestrated lung was found on pathological examination. This unusual combination of an extralobar pulmonary sequestration and a foregut cyst points to a common embryological pathogenesis.