A 53-year-old man was referred with the complaints of persistent cough ongoing for approximately two years with intervals, weakness and dyspnea on effort. Posteroanterior lung radiography showed normal lung parenchyma, except azygos fissure and lobe view. Fiber optic bronchoscopy demonstrated tracheal bronchus on the right lateral wall of trachea, 2.5-3 cm above the main carina. Thoracic computed tomography revealed a view in soft-tissue density located in the left lung posterior basal segment, sized 4x2.5 cm, which had an aberrant arterial supply originating from thoracic aorta and was consistent with intralobar pulmonary sequestration. We present this case with a literature review, as we did not find any other case having all these three anomalies together in the literature search.