Granular Cell Tumors(GCT) were originally described as myoblastic myomas. This tumor is believed to originate from Schwann cells based on subsequent scientific investigations. Although it usually appears in the head and neck, it can also appear in other organs as well. Endobronchial granular cell tumors are rather rare and should be differentiated from other common endobronchial diseases such as bronchogenic carcinoma and endobronchial tuberculosis, especially. A case of a patient with an extremely rare condition of endobronchial granular cell tumor concurrent with malignant mediastinal tumor is reported.