Pulmonary sequestration is a rare anomaly, in which a local area of a lung is supplied separately by an anomalous artery that arises from the aorta or one of its branches. Infection, mainly bacterial, is a major complication of sequestration. We report the case of a 17-year-old male patient, who presented with cough and fever. The contrast-enhanced chest computer tomomgraphy (CT) scans revealed an aberrant artery that originated from the descending thoracic aorta. He underwent a left-lower lobectomy. Macroscopically, the abnormal segment presented as multiple heterogenous cystic and solid lesions, and the cysts were filled with mucoid and pus-like material. Histology showed that the pulmonary parenchyma had been replaced by caseating epitheloid granulomas. The mycobacterial culture of his sputum was positive. On the basis of these results, the diagnosis of tuberculosis was established. The patient was treated with anti-tuberculous medication for 6 months, and 1 year later, his clinical status remained excellent.