Primary malignant tracheal tumors are not common and adenoid cystic carcinoma (ACC) of trachea is very rare. Hereby we discuss an extremely rare case of ACC of proximal trachea, which was diagnosed in a 60-year-old male who presents with recently diagnosed stridor and cough since 2 months which was ever misdiagnosed as chronic obstructive pulmonary disease (COPD). Fibreoptic bronchoscopy and computerised tomographic (CT) scan of chest revealed an endotracheal mass that was on histopathological examination showed adenoid cystic carcinoma of trachea. As per search in medline very few such cases are reported in india itself. The patient was treated by surgical sleeve resection followed by radiotherapy and is successfully on regular follow-up.