Inflammatory myofibroblastic tumors (IMT) of the lung are considered rare and benign; however, involvement of adjacent thoracic organs, local recurrence and distant metastases have been described. The potential presence of distant metastases supports the hypothesis that those tumors should not be considered 'clinically' benign, although histological features suggest this attitude; thus, complete resection and careful follow-up are mandatory. We present a case of a bilateral pulmonary IMT with left adrenal gland metastasis in a patient with dyspnea and cough.