Benign pulmonary tumors are rare entities, and among them bronchial lipomas are the most uncommon. Up to date, about 80 cases have been reported in the English literature. But, the bronchial lipoma with extrabronchial growth causing middle lobe syndrome and pneumonia is extremely rare. Bronchial lipomas, mainly arising from normal fatty tissue of the proximal portion of the lobar or segmental bronchi, are histologically benign. But if diagnosis and treatments are delayed, they can produce extensive pulmonary parenchymal damage and irreversible brochiectasis distally. So whenever possible, the treatment of choice is resection by means of bronchoscopy via early diagnosis. But if endoscopic removal is not possible because the distal end of the tumor could not be visualized by fiberoptic bronchoscopy or if the nature of the tumor is unclear, surgery is necessary, with lobectomy or pneumonectomy being required in most cases due to the extensively damaged pulmonary parenchyma. We present a case of bronchial lipoma with extrabronchial growth, with a review of the literature and report of an unusual case.