Although lipomas are common benign tumors in humans, endobronchial lipomas are quitely rare. Up to date, about 60 cases have been reported in the English literature. But endobronchial lipomas causing middle lobe syndrome were only 4 cases. These benign slow-growing tumors generally occur in the proximal portion of the lobar or segmental bronchi and originate from fatty tissue that is normally present in the bronchial tree. Smoking or chronic inflammation may be important in the pathogenesis of these tumors. Bronchoscopy is the definite diagnostic tool but, in general, open thoracotomy is required for diagnosis and treatment. Here, we present a case of endobronchial lipoma, with a review of the literature.