We report a rare case of an oncocytic mucoepidermoid carcinoma of the trachea, which presented in a 78-year-old woman with hemoptysis. Oncocytic cells comprised the majority of this low-grade lesion and demonstrated granular cytoplasmic phosphotungstic acid-hematoxylin staining as well as strong immunohistochemical reactivity to antimitochondrial antibody. Most tracheobronchial tumors with oncocytic change are carcinoid tumors. To our knowledge, this is the first oncocytic mucoepidermoid carcinoma of the trachea reported. This diagnosis was facilitated by histochemical and immunohistochemical studies.