Inflammatory pseudotumor is a rare clinical entity. We report here the case of a 14-year-old boy who underwent lung resection en bloc with the lower esophagus, the mediastinal pleura and the diaphragm for an inflammatory pseudotumor of the left lower lobe. Postoperative radiotherapy was administered. The patient is well at 9-year follow-up. Inflammatory pseudotumor may present a particularly aggressive behavior. Multimodality approach, including extensive surgical resection, may be necessary in dealing with this disease.