Primary adenoid cystic carcinoma of lung is an unusual thoracic neoplasm, which is considered as a slow-growing low-grade malignancy. The primary treatment for this tumor is surgery. The role of radiotherapy, chemotherapy, and targeted agents is less well defined. Here we report a case of inoperable adenoid cystic carcinoma of the lung in a 42-year-old male, presenting with an unusual aggressive behavior. The patient received radiotherapy and oral tyrosine kinase inhibitor imatinib with effective palliation.