Both bronchial leiomyoma and pulmonary sequestration are rare conditions, and to the best of our knowledge there are no reports of the two conditions coexisting. We report a female patient with bronchial leiomyoma with acquired pulmonary sequestration who presented with dyspnea, cough and purulent sputum. The patient had been treated for pneumonia at a local medical clinic. but was transferred to our clinic beacausr there was no clinical improvement. A 3-D computed tomography scan revealed a 1.5 cm sized mass near the distal portion of the left main bronchus and an anomalous artery arising from the aorta. The patient showed clinical improvement after a left lower lobectomy and a ligation of the anomalous artery.