Bronchopulmonary sequestration is an uncommon anomaly in children. It is characterized by nonfunctioning pulmonary tissue that is not connected to the normal tracheobronchial tree, and its blood supply derived from a systemic arterial source. A 2900-gram male term baby was referred to our clinic with the diagnosis of anal atresia. A diverting sigmoidostomy was performed. He was started to oral feeding on the second postoperative day, but patient’s general condition was impaired and right lower lobe pneumonia was documented on chest X-ray. A thoracic CT scan demonstrated multiple cysts and pneumonic consolidation, which indicated abscess at right lower lobe. The thoracic CT scan also showed bronchus like structure, which was entering to the esophagus. An esophagogram showed a fistula from distal esophagus to the right lower lobe of the lung. At thoracotomy, a connection between the right lower lobe of the lung and distal of the esophagus by its bronchus was noted. An anomalous artery was found, originating from thoracic aorta. The fistula was divided from esophagus and right lower lobectomy was performed. Also basal region of the right middle lobe were not inflated with ventilation and congested with a clear demarcation from normal appearing lung. This situation was evaluated as an intralobar sequestration and resection was performed. We present here association of communicating extralobar sequestration with intralobar sequestration in a patient who had anal atresia. To the best of our knowledge this combination has never been reported before.