Granular cell myoblastoma is a common lesion of uncertain histogenesis. It commonly affects the tongue, breast, and subcutaneous tissues. However, its occurrence in the tracheobronchial tree is rare. Although generally a benign lesion, isolated malignant granular cell myoblastoma as well as its coexistence with other primary bronchogenic carcinomas has been documented. In spite of recent anecdotal reports advocating endoscopic removal of this lesion, we believe definitive surgical excision is a more rational choice of treatment.