Inflammatory pseudotumor of the lung is considered to be a rare, benign, neoplastic lesion, consisting mainly of spindle mesenchymal cells, sometimes in such a way that its histological appearance mimics that of a spindle cell sarcoma, fibrous histiocytoma or fibrosarcoma. On the occasion of a case managed in our department, the literature is reviewed, in an attempt to clarify some issues concerning this tumor. Emphasis is given to complete resection of the tumor for both diagnostic and therapeutic purposes. Malignant behavior may occur and recurrence is possible.