Glomus tumors are uncommon tumors that are originated from smooth muscle cells of the neuromioarterials glomus bodies located in the arteriovenous anastomoses subcutaneous tissue or deep dermis of the extremities, mainly in the palms of the hands, wrists and subungual areas of the fingers. Carcinoid tumor, as the glomus tumor, can show an organoid pattern, increased vascularity, and uniform, round cells with eosinophilic cytoplasm, but usually are positive for cytokeratin and always stained with chromogranin and synaptophysin showing negative for smooth muscle markers which is presented in our case. Glomus tumors have a good prognosis and surgical resection is the treatment of choice. In our case, the patient underwent pulmonary bilobectomy because of the location of the tumor in the transition between the middle lobe and the basal bronchial trunk right lower lobe divisions. It is presented thus a glomus tumor with exceptional localization (pulmonary and bronchial) of benign histological features, according to most of the cases reported in the literature emphasizing their particular rare location, histological, and immunohistochemical profile, which helps the differential diagnosis with other most common tumors of bronchial location.