Granular cell tumor (GCT) is a rare benign mesenchymal tumor that uncommonly occurs in the lung and tracheobronchial tree. Small cell carcinoma of lung is a centrally located malignant neoplasm that commonly occurs in elderly smokers. Concomitant existence of both the neoplasm in lung is extremely rare with only one reported case in the literature. Few rare combinations of GCT with other primary bronchogenic carcinomas have also been reported. Clinical symptoms depend upon the site and size of the tumor. Definitive diagnosis is by histopathological and proper immunohistochemical analysis. Identification of this entity is important as treatment requires individual therapy protocols that depend on the presence of metastasis, location of the tumors, and type of bronchogenic carcinoma.